Correlation between the frequency difference limen and an index based on principal component analysis of the frequency-following response of normal hearing listeners

Publication date: Available online 10 December 2016
Source:Hearing Research
Author(s): Xiaochen Zhang, Qin Gong
Subcortical phase locking tends to reflect performance differences in tasks related to pitch perception across different types of populations. Enhancement or attenuation in its strength may correspond to population excellence or deficiency in pitch perception. However, it is still unclear whether differences in perceptual capability among individuals with normal hearing can be predicted by subcortical phase locking. In this study, we examined the brain-behavior relationship between frequency-following responses (FFRs) evoked by pure/sweeping tones and frequency difference limens (FDLs). FFRs are considered to reflect subcortical phase locking, and FDLs are a psychophysical measure of behavioral performance in pitch discrimination. Traditional measures of FFR strength were found to be poorly correlated with FDL. Here, we introduced principal component analysis into FFR analysis and extracted an FFR component that was correlated with individual pitch discrimination. The absolute value of the score of this FFR principal component (but not the original score) was negatively correlated with FDL, regardless of stimulus type. The topographic distribution of this component was relatively constant across individuals and across stimulus types, and the inferior colliculus was identified as its origin. The findings suggest that subcortical phase locking at certain but not all FFR generators carries the neural information required for the prediction of individual pitch perception among humans with normal hearing.



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Correlation between the frequency difference limen and an index based on principal component analysis of the frequency-following response of normal hearing listeners

Publication date: Available online 10 December 2016
Source:Hearing Research
Author(s): Xiaochen Zhang, Qin Gong
Subcortical phase locking tends to reflect performance differences in tasks related to pitch perception across different types of populations. Enhancement or attenuation in its strength may correspond to population excellence or deficiency in pitch perception. However, it is still unclear whether differences in perceptual capability among individuals with normal hearing can be predicted by subcortical phase locking. In this study, we examined the brain-behavior relationship between frequency-following responses (FFRs) evoked by pure/sweeping tones and frequency difference limens (FDLs). FFRs are considered to reflect subcortical phase locking, and FDLs are a psychophysical measure of behavioral performance in pitch discrimination. Traditional measures of FFR strength were found to be poorly correlated with FDL. Here, we introduced principal component analysis into FFR analysis and extracted an FFR component that was correlated with individual pitch discrimination. The absolute value of the score of this FFR principal component (but not the original score) was negatively correlated with FDL, regardless of stimulus type. The topographic distribution of this component was relatively constant across individuals and across stimulus types, and the inferior colliculus was identified as its origin. The findings suggest that subcortical phase locking at certain but not all FFR generators carries the neural information required for the prediction of individual pitch perception among humans with normal hearing.



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Correlation between the frequency difference limen and an index based on principal component analysis of the frequency-following response of normal hearing listeners

Publication date: Available online 10 December 2016
Source:Hearing Research
Author(s): Xiaochen Zhang, Qin Gong
Subcortical phase locking tends to reflect performance differences in tasks related to pitch perception across different types of populations. Enhancement or attenuation in its strength may correspond to population excellence or deficiency in pitch perception. However, it is still unclear whether differences in perceptual capability among individuals with normal hearing can be predicted by subcortical phase locking. In this study, we examined the brain-behavior relationship between frequency-following responses (FFRs) evoked by pure/sweeping tones and frequency difference limens (FDLs). FFRs are considered to reflect subcortical phase locking, and FDLs are a psychophysical measure of behavioral performance in pitch discrimination. Traditional measures of FFR strength were found to be poorly correlated with FDL. Here, we introduced principal component analysis into FFR analysis and extracted an FFR component that was correlated with individual pitch discrimination. The absolute value of the score of this FFR principal component (but not the original score) was negatively correlated with FDL, regardless of stimulus type. The topographic distribution of this component was relatively constant across individuals and across stimulus types, and the inferior colliculus was identified as its origin. The findings suggest that subcortical phase locking at certain but not all FFR generators carries the neural information required for the prediction of individual pitch perception among humans with normal hearing.



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Correlation between the frequency difference limen and an index based on principal component analysis of the frequency-following response of normal hearing listeners

Publication date: Available online 10 December 2016
Source:Hearing Research
Author(s): Xiaochen Zhang, Qin Gong
Subcortical phase locking tends to reflect performance differences in tasks related to pitch perception across different types of populations. Enhancement or attenuation in its strength may correspond to population excellence or deficiency in pitch perception. However, it is still unclear whether differences in perceptual capability among individuals with normal hearing can be predicted by subcortical phase locking. In this study, we examined the brain-behavior relationship between frequency-following responses (FFRs) evoked by pure/sweeping tones and frequency difference limens (FDLs). FFRs are considered to reflect subcortical phase locking, and FDLs are a psychophysical measure of behavioral performance in pitch discrimination. Traditional measures of FFR strength were found to be poorly correlated with FDL. Here, we introduced principal component analysis into FFR analysis and extracted an FFR component that was correlated with individual pitch discrimination. The absolute value of the score of this FFR principal component (but not the original score) was negatively correlated with FDL, regardless of stimulus type. The topographic distribution of this component was relatively constant across individuals and across stimulus types, and the inferior colliculus was identified as its origin. The findings suggest that subcortical phase locking at certain but not all FFR generators carries the neural information required for the prediction of individual pitch perception among humans with normal hearing.



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Correlation between the frequency difference limen and an index based on principal component analysis of the frequency-following response of normal hearing listeners

Publication date: Available online 10 December 2016
Source:Hearing Research
Author(s): Xiaochen Zhang, Qin Gong
Subcortical phase locking tends to reflect performance differences in tasks related to pitch perception across different types of populations. Enhancement or attenuation in its strength may correspond to population excellence or deficiency in pitch perception. However, it is still unclear whether differences in perceptual capability among individuals with normal hearing can be predicted by subcortical phase locking. In this study, we examined the brain-behavior relationship between frequency-following responses (FFRs) evoked by pure/sweeping tones and frequency difference limens (FDLs). FFRs are considered to reflect subcortical phase locking, and FDLs are a psychophysical measure of behavioral performance in pitch discrimination. Traditional measures of FFR strength were found to be poorly correlated with FDL. Here, we introduced principal component analysis into FFR analysis and extracted an FFR component that was correlated with individual pitch discrimination. The absolute value of the score of this FFR principal component (but not the original score) was negatively correlated with FDL, regardless of stimulus type. The topographic distribution of this component was relatively constant across individuals and across stimulus types, and the inferior colliculus was identified as its origin. The findings suggest that subcortical phase locking at certain but not all FFR generators carries the neural information required for the prediction of individual pitch perception among humans with normal hearing.



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Acoustic neuroma as first sign of inner ear functional disorders.

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Acoustic neuroma as first sign of inner ear functional disorders.

Otolaryngol Pol. 2016 Oct 31;70(5):19-25

Authors: Bielińska M, Owczarek K, Nowosielska-Grygiel J, Olszewski J, Pietkiewicz P

Abstract
INTRODUCTION: The aim of this work was to describe acoustic neuromas as the first signs of inner ear functional disorders.
MATERIAL AND METHODS: The study covered 3,456 audiological and otoneurological patients, who were treated in the Department of Otolaryngology, Laryngological Oncology, Audiology and Phoniatrics Military Medical Academy University Teaching Hospital in Lodz within the period of 2011-2016. Among the studied subjects, an acoustic neuroma on the vestibulocochlear nerve was diagnosed in 13 cases (5,16%), including 9 women and 4 men. Each patient underwent a medical interview and an objective examination with static and dynamic tests, pure tone audiometry and speech audiometry tests, impedance audiometry tests, characteristics of tinnitus, videonystagmography and auditory brainstem evoked potentials (ABR) in crack. In each case of an incorrect ABR recording, contrast-enhanced MRI was performed.
STUDY RESULTS: The conducted tests showed an incorrect ABR recording in 252 patients (7,29%, including 54,37% women and 45,63% men). In the performed contrast-enhanced MRI, 13 patients (5,16%), including 3,57% women and 1,59% men, had an image typical to neuroma of the vestibulocochlear nerve. Each one of the studied patients showed signs and symptoms typical to an acoustic neuroma such as hypoacusia and balance disorders, tinnitus in 12 subjects, headaches in the temporal and occipital area in 4 subjects, trigeminal neuralgia symptoms in 2 subjects and vision problems like scotoma and blurred vision reported by 1 patient.
CONCLUSION: Each case of sensorineural hearing loss, particularly unilateral sensorineural hearing loss, requires expanded hearing tests and auditory brainstem evoked potentials tests. An incorrect brainstem evoked potentials recording such as: an elongated latency of the 5th wave, an elongated interlatency of 1-2-3 and 1-2-3-4 waves, requires a contract-enhanced magnetic resonance imaging to diagnose cerebellopontine angle tumours.

PMID: 27935543 [PubMed - in process]

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Acoustic neuroma as first sign of inner ear functional disorders.

Related Articles

Acoustic neuroma as first sign of inner ear functional disorders.

Otolaryngol Pol. 2016 Oct 31;70(5):19-25

Authors: Bielińska M, Owczarek K, Nowosielska-Grygiel J, Olszewski J, Pietkiewicz P

Abstract
INTRODUCTION: The aim of this work was to describe acoustic neuromas as the first signs of inner ear functional disorders.
MATERIAL AND METHODS: The study covered 3,456 audiological and otoneurological patients, who were treated in the Department of Otolaryngology, Laryngological Oncology, Audiology and Phoniatrics Military Medical Academy University Teaching Hospital in Lodz within the period of 2011-2016. Among the studied subjects, an acoustic neuroma on the vestibulocochlear nerve was diagnosed in 13 cases (5,16%), including 9 women and 4 men. Each patient underwent a medical interview and an objective examination with static and dynamic tests, pure tone audiometry and speech audiometry tests, impedance audiometry tests, characteristics of tinnitus, videonystagmography and auditory brainstem evoked potentials (ABR) in crack. In each case of an incorrect ABR recording, contrast-enhanced MRI was performed.
STUDY RESULTS: The conducted tests showed an incorrect ABR recording in 252 patients (7,29%, including 54,37% women and 45,63% men). In the performed contrast-enhanced MRI, 13 patients (5,16%), including 3,57% women and 1,59% men, had an image typical to neuroma of the vestibulocochlear nerve. Each one of the studied patients showed signs and symptoms typical to an acoustic neuroma such as hypoacusia and balance disorders, tinnitus in 12 subjects, headaches in the temporal and occipital area in 4 subjects, trigeminal neuralgia symptoms in 2 subjects and vision problems like scotoma and blurred vision reported by 1 patient.
CONCLUSION: Each case of sensorineural hearing loss, particularly unilateral sensorineural hearing loss, requires expanded hearing tests and auditory brainstem evoked potentials tests. An incorrect brainstem evoked potentials recording such as: an elongated latency of the 5th wave, an elongated interlatency of 1-2-3 and 1-2-3-4 waves, requires a contract-enhanced magnetic resonance imaging to diagnose cerebellopontine angle tumours.

PMID: 27935543 [PubMed - in process]

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Identification of a novel collagen type IV alpha-4 (COL4A4) mutation in a Chinese family with autosomal dominant Alport syndrome using exome sequencing.

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Identification of a novel collagen type IV alpha-4 (COL4A4) mutation in a Chinese family with autosomal dominant Alport syndrome using exome sequencing.

Indian J Med Res. 2016 Aug;144(2):200-205

Authors: Deng S, Xu H, Yuan J, Xiao J, Yuan L, Deng X, Guan L, Zhu A, Rong P, Zhang J, Deng H

Abstract
BACKGROUND & OBJECTIVES: Alport syndrome (AS) is an inherited disorder characterized by glomerulonephritis and end-stage renal disease (ESRD). The aim of this study was to identify the gene responsible for the glomerulopathy in a Chinese family with autosomal dominant AS using exome sequencing.
METHODS: A 4-generation, 30-member Chinese Han family was enrolled in this study. Exome sequencing was conducted in the proband of the family, and then direct sequencing was performed in family members of the pedigree and 100 normal controls.
RESULTS: A novel frameshift mutation, c.3213delA (p.Gly1072GlufsFNx0169), in the collagen type IV alpha-4 gene (COL4A4) was found to be the genetic cause. Neither sensorineural hearing loss nor ocular abnormalities were present in the patients of this family. Other clinical features, such as age of onset, age of ESRD occurring and disease severity, varied among the patients of this family.
INTERPRETATION & CONCLUSIONS: A novel frameshift mutation, c.3213delA (p.Gly1072GlufsFNx0169) in the COL4A4 gene, was identified in the Chinese pedigree with autosomal dominant AS. Our findings may provide new insights into the cause and diagnosis of AS and also have implications for genetic counselling.

PMID: 27934798 [PubMed - in process]

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Pain, not structural impairments may explain activity limitations in people with gluteal tendinopathy or hip osteoarthritis: A cross sectional study

Publication date: February 2017
Source:Gait & Posture, Volume 52
Author(s): Angela Fearon, Teresa Neeman, Paul Smith, Jennie Scarvell, Jill Cook
QuestionWhat are the functional differences between people with greater trochanteric pain syndrome (GT), hip osteoarthritis (OA) or an asymptomatic population as measured by walking, Time Up and Go, single leg standing and strength?DesignCross sectional study with blinded measurers.Participants38 participants with GT, 20 with end stage hip OA and 21 asymptomatic healthy control (AS) participants. All participants were women.Outcome measuresPain (numeric rating scale), Walking speed (m/s), cadence (steps/min) and step length (m) measured via the 10m walk test and the Timed Up and Go; balance via single leg stance (s) duration; and hip abduction, adduction, medial and lateral rotation strength, standardized to body mass (BM) via the body mass average index (BMavg), measured via a wall mounted dynamometer.ResultsThe two symptomatic groups reported similar pain levels (p=0.226), more pain then the AS group (p<0.000). Compared to the AS participants, participants with GT or hip OA demonstrated lower walking speed (10mwt and TUG, p<0.001), lower cadence and shorter duration single leg stance on the affected leg (p<0.05). Participants with GT or hip OA also demonstrated bilaterally weaker hip abduction than the AS group (p≤0.005). Compared to AS and GT participants, participants with hip OA demonstrated adduction weakness on the affected side (p=0.008 and p=0.002 respectively).ConclusionThere is a significant level of dysfunction and impairments associated with GT and hip OA. As activity limitations do not appear to be differentiated by structural impairments, we suggest that pain, rather than the underlying pathology may be the driving impairment that leads to walking and single leg standing dysfunction.



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Pain, not structural impairments may explain activity limitations in people with gluteal tendinopathy or hip osteoarthritis: A cross sectional study

Publication date: February 2017
Source:Gait & Posture, Volume 52
Author(s): Angela Fearon, Teresa Neeman, Paul Smith, Jennie Scarvell, Jill Cook
QuestionWhat are the functional differences between people with greater trochanteric pain syndrome (GT), hip osteoarthritis (OA) or an asymptomatic population as measured by walking, Time Up and Go, single leg standing and strength?DesignCross sectional study with blinded measurers.Participants38 participants with GT, 20 with end stage hip OA and 21 asymptomatic healthy control (AS) participants. All participants were women.Outcome measuresPain (numeric rating scale), Walking speed (m/s), cadence (steps/min) and step length (m) measured via the 10m walk test and the Timed Up and Go; balance via single leg stance (s) duration; and hip abduction, adduction, medial and lateral rotation strength, standardized to body mass (BM) via the body mass average index (BMavg), measured via a wall mounted dynamometer.ResultsThe two symptomatic groups reported similar pain levels (p=0.226), more pain then the AS group (p<0.000). Compared to the AS participants, participants with GT or hip OA demonstrated lower walking speed (10mwt and TUG, p<0.001), lower cadence and shorter duration single leg stance on the affected leg (p<0.05). Participants with GT or hip OA also demonstrated bilaterally weaker hip abduction than the AS group (p≤0.005). Compared to AS and GT participants, participants with hip OA demonstrated adduction weakness on the affected side (p=0.008 and p=0.002 respectively).ConclusionThere is a significant level of dysfunction and impairments associated with GT and hip OA. As activity limitations do not appear to be differentiated by structural impairments, we suggest that pain, rather than the underlying pathology may be the driving impairment that leads to walking and single leg standing dysfunction.



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Pain, not structural impairments may explain activity limitations in people with gluteal tendinopathy or hip osteoarthritis: A cross sectional study

Publication date: February 2017
Source:Gait & Posture, Volume 52
Author(s): Angela Fearon, Teresa Neeman, Paul Smith, Jennie Scarvell, Jill Cook
QuestionWhat are the functional differences between people with greater trochanteric pain syndrome (GT), hip osteoarthritis (OA) or an asymptomatic population as measured by walking, Time Up and Go, single leg standing and strength?DesignCross sectional study with blinded measurers.Participants38 participants with GT, 20 with end stage hip OA and 21 asymptomatic healthy control (AS) participants. All participants were women.Outcome measuresPain (numeric rating scale), Walking speed (m/s), cadence (steps/min) and step length (m) measured via the 10m walk test and the Timed Up and Go; balance via single leg stance (s) duration; and hip abduction, adduction, medial and lateral rotation strength, standardized to body mass (BM) via the body mass average index (BMavg), measured via a wall mounted dynamometer.ResultsThe two symptomatic groups reported similar pain levels (p=0.226), more pain then the AS group (p<0.000). Compared to the AS participants, participants with GT or hip OA demonstrated lower walking speed (10mwt and TUG, p<0.001), lower cadence and shorter duration single leg stance on the affected leg (p<0.05). Participants with GT or hip OA also demonstrated bilaterally weaker hip abduction than the AS group (p≤0.005). Compared to AS and GT participants, participants with hip OA demonstrated adduction weakness on the affected side (p=0.008 and p=0.002 respectively).ConclusionThere is a significant level of dysfunction and impairments associated with GT and hip OA. As activity limitations do not appear to be differentiated by structural impairments, we suggest that pain, rather than the underlying pathology may be the driving impairment that leads to walking and single leg standing dysfunction.



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Optimizations for the Electrically-Evoked Stapedial Reflex Threshold Measurement in Cochlear Implant Recipients.

Objective: The electrically-evoked stapedial reflex threshold (eSRT) has proven to be useful in setting upper stimulation levels of cochlear implant recipients. However, the literature suggests that the reflex can be difficult to observe in a significant percentage of the population. The primary goal of this investigation was to assess the difference in eSRT levels obtained with alternative acoustic admittance probe tone frequencies. Design: A repeated-measures design was used to examine the effect of 3 probe tone frequencies (226, 678, and 1000 Hz) on eSRT in 23 adults with cochlear implants. Results: The mean eSRT measured using the conventional probe tone of 226 Hz was significantly higher than the mean eSRT measured with use of 678 and 1000 Hz probe tones. The mean eSRT were 174, 167, and 165 charge units with use of 226, 678, and 1000 Hz probe tones, respectively. There was not a statistically significant difference between the average eSRTs for probe tones 678 and 1000 Hz. Twenty of 23 participants had eSRT at lower charge unit levels with use of either a 678 or 1000 Hz probe tone when compared with the 226 Hz probe tone. Two participants had eSRT measured with 678 or 1000 Hz probe tones that were equal in level to the eSRT measured with a 226 Hz probe tone. Only 1 participant had an eSRT that was obtained at a lower charge unit level with a 226 Hz probe tone relative to the eSRT obtained with a 678 and 1000 Hz probe tone. Conclusions: The results of this investigation demonstrate that the use of a standard 226 Hz probe tone is not ideal for measurement of the eSRT. The use of higher probe tone frequencies (i.e., 678 or 1000 Hz) resulted in lower eSRT levels when compared with the eSRT levels obtained with use of a 226 probe tone. In addition, 4 of the 23 participants included in this study did not have a measureable eSRT with use of a 226 Hz probe tone, but all of the participants had measureable eSRT with use of both the 678 and 1000 Hz probe tones. Additional work is required to understand the clinical implication of these changes in the context of cochlear implant programming. Copyright (C) 2016 Wolters Kluwer Health, Inc. All rights reserved.

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Dichotic Digits Test Performance Across the Ages: Results From Two Large Epidemiologic Cohort Studies.

Objectives: The Dichotic Digits test (DDT) has been widely used to assess central auditory processing but there is limited information on observed DDT performance in a general population. The purpose of the study was to determine factors related to DDT performance in a large cohort spanning the adult age range. Design: The study was cross-sectional and subjects were participants in the Epidemiology of Hearing Loss Study (EHLS), a population-based investigation of age-related hearing loss, or the Beaver Dam Offspring Study (BOSS), a study of aging in the adult offspring of the EHLS members. Subjects seen during the 4th EHLS (2008 to 2010) or the 2nd BOSS (2010 to 2013) examination were included (N = 3655 participants [1391 EHLS, 2264 BOSS]; mean age = 61.1 years, range = 21 to 100 years). The free and right ear-directed recall DDTs were administered using 25 sets of triple-digit pairs with a 70 dB HL presentation level. Pure-tone audiometric testing was conducted and the pure-tone threshold average (PTA) at 0.5, 1, 2, and 4 kHz was categorized using the worse ear: no loss = PTA 40 dB HL. Cognitive impairment was defined as a Mini-Mental State Examination score

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Optimizations for the Electrically-Evoked Stapedial Reflex Threshold Measurement in Cochlear Implant Recipients.

Objective: The electrically-evoked stapedial reflex threshold (eSRT) has proven to be useful in setting upper stimulation levels of cochlear implant recipients. However, the literature suggests that the reflex can be difficult to observe in a significant percentage of the population. The primary goal of this investigation was to assess the difference in eSRT levels obtained with alternative acoustic admittance probe tone frequencies. Design: A repeated-measures design was used to examine the effect of 3 probe tone frequencies (226, 678, and 1000 Hz) on eSRT in 23 adults with cochlear implants. Results: The mean eSRT measured using the conventional probe tone of 226 Hz was significantly higher than the mean eSRT measured with use of 678 and 1000 Hz probe tones. The mean eSRT were 174, 167, and 165 charge units with use of 226, 678, and 1000 Hz probe tones, respectively. There was not a statistically significant difference between the average eSRTs for probe tones 678 and 1000 Hz. Twenty of 23 participants had eSRT at lower charge unit levels with use of either a 678 or 1000 Hz probe tone when compared with the 226 Hz probe tone. Two participants had eSRT measured with 678 or 1000 Hz probe tones that were equal in level to the eSRT measured with a 226 Hz probe tone. Only 1 participant had an eSRT that was obtained at a lower charge unit level with a 226 Hz probe tone relative to the eSRT obtained with a 678 and 1000 Hz probe tone. Conclusions: The results of this investigation demonstrate that the use of a standard 226 Hz probe tone is not ideal for measurement of the eSRT. The use of higher probe tone frequencies (i.e., 678 or 1000 Hz) resulted in lower eSRT levels when compared with the eSRT levels obtained with use of a 226 probe tone. In addition, 4 of the 23 participants included in this study did not have a measureable eSRT with use of a 226 Hz probe tone, but all of the participants had measureable eSRT with use of both the 678 and 1000 Hz probe tones. Additional work is required to understand the clinical implication of these changes in the context of cochlear implant programming. Copyright (C) 2016 Wolters Kluwer Health, Inc. All rights reserved.

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Dichotic Digits Test Performance Across the Ages: Results From Two Large Epidemiologic Cohort Studies.

Objectives: The Dichotic Digits test (DDT) has been widely used to assess central auditory processing but there is limited information on observed DDT performance in a general population. The purpose of the study was to determine factors related to DDT performance in a large cohort spanning the adult age range. Design: The study was cross-sectional and subjects were participants in the Epidemiology of Hearing Loss Study (EHLS), a population-based investigation of age-related hearing loss, or the Beaver Dam Offspring Study (BOSS), a study of aging in the adult offspring of the EHLS members. Subjects seen during the 4th EHLS (2008 to 2010) or the 2nd BOSS (2010 to 2013) examination were included (N = 3655 participants [1391 EHLS, 2264 BOSS]; mean age = 61.1 years, range = 21 to 100 years). The free and right ear-directed recall DDTs were administered using 25 sets of triple-digit pairs with a 70 dB HL presentation level. Pure-tone audiometric testing was conducted and the pure-tone threshold average (PTA) at 0.5, 1, 2, and 4 kHz was categorized using the worse ear: no loss = PTA 40 dB HL. Cognitive impairment was defined as a Mini-Mental State Examination score

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Optimizations for the Electrically-Evoked Stapedial Reflex Threshold Measurement in Cochlear Implant Recipients.

Objective: The electrically-evoked stapedial reflex threshold (eSRT) has proven to be useful in setting upper stimulation levels of cochlear implant recipients. However, the literature suggests that the reflex can be difficult to observe in a significant percentage of the population. The primary goal of this investigation was to assess the difference in eSRT levels obtained with alternative acoustic admittance probe tone frequencies. Design: A repeated-measures design was used to examine the effect of 3 probe tone frequencies (226, 678, and 1000 Hz) on eSRT in 23 adults with cochlear implants. Results: The mean eSRT measured using the conventional probe tone of 226 Hz was significantly higher than the mean eSRT measured with use of 678 and 1000 Hz probe tones. The mean eSRT were 174, 167, and 165 charge units with use of 226, 678, and 1000 Hz probe tones, respectively. There was not a statistically significant difference between the average eSRTs for probe tones 678 and 1000 Hz. Twenty of 23 participants had eSRT at lower charge unit levels with use of either a 678 or 1000 Hz probe tone when compared with the 226 Hz probe tone. Two participants had eSRT measured with 678 or 1000 Hz probe tones that were equal in level to the eSRT measured with a 226 Hz probe tone. Only 1 participant had an eSRT that was obtained at a lower charge unit level with a 226 Hz probe tone relative to the eSRT obtained with a 678 and 1000 Hz probe tone. Conclusions: The results of this investigation demonstrate that the use of a standard 226 Hz probe tone is not ideal for measurement of the eSRT. The use of higher probe tone frequencies (i.e., 678 or 1000 Hz) resulted in lower eSRT levels when compared with the eSRT levels obtained with use of a 226 probe tone. In addition, 4 of the 23 participants included in this study did not have a measureable eSRT with use of a 226 Hz probe tone, but all of the participants had measureable eSRT with use of both the 678 and 1000 Hz probe tones. Additional work is required to understand the clinical implication of these changes in the context of cochlear implant programming. Copyright (C) 2016 Wolters Kluwer Health, Inc. All rights reserved.

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Dichotic Digits Test Performance Across the Ages: Results From Two Large Epidemiologic Cohort Studies.

Objectives: The Dichotic Digits test (DDT) has been widely used to assess central auditory processing but there is limited information on observed DDT performance in a general population. The purpose of the study was to determine factors related to DDT performance in a large cohort spanning the adult age range. Design: The study was cross-sectional and subjects were participants in the Epidemiology of Hearing Loss Study (EHLS), a population-based investigation of age-related hearing loss, or the Beaver Dam Offspring Study (BOSS), a study of aging in the adult offspring of the EHLS members. Subjects seen during the 4th EHLS (2008 to 2010) or the 2nd BOSS (2010 to 2013) examination were included (N = 3655 participants [1391 EHLS, 2264 BOSS]; mean age = 61.1 years, range = 21 to 100 years). The free and right ear-directed recall DDTs were administered using 25 sets of triple-digit pairs with a 70 dB HL presentation level. Pure-tone audiometric testing was conducted and the pure-tone threshold average (PTA) at 0.5, 1, 2, and 4 kHz was categorized using the worse ear: no loss = PTA 40 dB HL. Cognitive impairment was defined as a Mini-Mental State Examination score

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Nystagmus at the Onset of Vertiginous Attack in Ménière's Disease

Objective: To clarify the direction and characteristics of nystagmus at the onset of a vertiginous attack in Ménière's disease. Patients: Two patients with Ménière's disease, whose nystagmus at the onset of a vertiginous attack was recorded using electronystagmography. Interventions: Diagnostic. Main Outcome Measures: Electronystagmographic recordings of nystagmus. Results: In both patients, nystagmus was directed toward the affected side over the entire course of the vertiginous attack. One patient experienced a severe sensation of vertigo and exhibited strong nystagmus from the onset of the attack. The other patient reported a mild sensation of vertigo, which was accompanied by intermittent nystagmus. Conclusions: Vertiginous attacks in Ménière's disease are accompanied by irritative nystagmus. The intensity and characteristics (e.g., continuous or intermittent expression) of the nystagmus may be associated with pathophysiological severity.

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Comparison of Speech-in-Noise and Localization Benefits in Unilateral Hearing Loss Subjects Using Contralateral Routing of Signal Hearing Aids or Bone-Anchored Implants

Objective: To compare the benefit of wireless contralateral routing of signal (CROS) technology to bone-anchored implant (BAI) technology in monaural listeners. Study Design: Prospective, single-subject. Setting: Tertiary academic referral center. Patients: Adult English speaking subjects using either a CROS hearing aid or BAI as treatment for unilateral severe-profound hearing loss. Interventions: Aided performance utilizing the subjects BAI or CROS hearing device. Main Outcome Measures: Outcome measures included speech-in-noise perception using the QuickSIN™ (Etymotic Research, Elkgrove Village, IL, 2001) speech-in-noise test and localization ability using narrow and broadband stimuli. Performance was measured in the unaided and aided condition and compared with normal hearing controls. Subjective outcomes measures included the Speech Spatial and Qualities hearing scale and the Glasgow Hearing Aid Benefit Profile. Results: A significant improvement in speech-in-noise performance for monaural listeners (p 

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History of the Endolymphatic Sac: From Anatomy to Surgery

Objectives: To evaluate the historical descriptive origins of the anatomy of endolymphatic sac (ELS), its initial dilation description, its association with tumors, and lastly its surgical implication. Methods: A collection of historical and contemporary references, and selected textbooks were compiled spanning the 18th century to present day with specific focus on the description of the ELS, ELS dilation, and ELS tumors (ELSTs). Results: Domenico Cotugno describes the ELS in 1760. Carl Hasse introduces the term endolymphatic sac (saccus endolymphaticus) in 1873, which has remained in use till today. Carlo Mondini, in 1791, describes ELS dilation. Georges Portmann proposes opening the ELS for the treatment of Ménière's disease (MD) in 1926. In 1984, the first reported case of an ELST is described with pathology consisting of an adenoma. Four years later, in 1988, the first report is published suggesting ELSTs in direct association to von Hippel-Lindau disease. Conclusion: Domenico Cotugno is precise in describing the ELS in 1760. A timeline of important events relating to the ELS is presented. These include ELS dilation, surgical opening of the ELS for Meniere's, and ELSTs relating to von Hippel-Lindau disease.

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Hearing Changes After Intratympanically Applied Steroids for Primary Therapy of Sudden Hearing Loss: A Meta-analysis Using Mathematical Simulations of Drug Delivery Protocols

Objective: Controlled and uncontrolled studies with primary intratympanic or combined intratympanic and systemic application of glucocorticosteroids for idiopathic sudden hearing loss were analyzed by means of a meta-analysis in an attempt to establish optimal local drug delivery protocols. Study Design: A total of 25 studies with 28 treatment groups between January 2000 and June 2014 were selected that adequately described drug delivery protocols. Cochlear drug levels were calculated by a validated computer model of drug dispersion in the inner ear fluids based on the concentration and volume of glucocorticoids applied, the time the drug remained in the middle ear, and the specific timing of injections. Various factors were compared with hearing outcome, including baseline data, individual parameters of the application protocols, calculated peak concentration (Cmax), and total dose (area under the curve). Results: There was no dependence of hearing outcome on individual parameters of the application protocol, Cmax, or area under the curve. Final hearing threshold was notably independent of delay of treatment. Conclusion: During primary intratympanic or combined steroid therapy of idiopathic sudden hearing loss, the tendency toward early treatment having a positive effect on hearing improvement is thought to be a “sham effect,” likely related to spontaneous recovery. Change in pure-tone average may not be an adequate outcome parameter to assess effectiveness of the intervention, as it depends on the degree of initial hearing loss. Final pure-tone average provides a better alternative.

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Hemifacial Spasm Secondary to Parotid Pleomorphic Adenoma With Stylomastoid Foramen Extension

Objective: To report hemifacial spasm (HFS) as a rare presenting symptom of a benign parotid neoplasm involving the stylomastoid foramen. Methods: Case report with literature review. Results: An 18-year-old woman presented with a 1-year history of a progressively enlarging right infra-auricular mass with new onset ipsilateral severe HFS. Clinical vetting unveiled a palpable 2 cm, firm, minimally mobile mass medial to the angle of the mandible. Imaging revealed a heterogeneous intraparotid mass with tumor extension into the stylomastoid foramen. Diagnostic consideration was given to neurogenic tumors and neoplasms of parotid origin. The patient ultimately underwent superficial parotidectomy and the tumor was found superficial and extrinsic to the proximal facial nerve trunk but extended into the stylomastoid foramen. The tumor was removed after performing mastoidectomy with mastoid segment facial nerve decompression. Frozen section pathology was consistent with pleomorphic adenoma. Postoperatively the patient had normal facial nerve function with immediate and complete resolution of HFS. To date there are only three other published reports of benign parotid tumors presenting with HFS and this is the first illustrating intratemporal tumor extension. Conclusions: HFS is a rare presenting symptom associated with benign parotid neoplasms with scarce documentation in the literature. In this index case, tumor extirpation with mastoid segment facial nerve decompression provided immediate and durable resolution of symptoms.

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Reduction of the Harmonic Series Influences Musical Enjoyment With Cochlear Implants

Objective: Cochlear implantation is associated with poor music perception and enjoyment. Reducing music complexity has been shown to enhance music enjoyment in cochlear implant (CI) recipients. In this study, we assess the impact of harmonic series reduction on music enjoyment. Study Design: Prospective analysis of music enjoyment in normal-hearing (NH) individuals and CI recipients. Setting: Single tertiary academic medical center. Patients: NH adults (N = 20) and CI users (N = 8) rated the Happy Birthday song on three validated enjoyment modalities—musicality, pleasantness, and naturalness. Intervention: Subjective rating of music excerpts. Main Outcome Measures: Participants listened to seven different instruments play the melody, each with five levels of harmonic reduction (Full, F3+F2+F1+F0, F2+F1+F0, F1+F0, F0). NH participants listened to the segments both with and without CI simulation. Linear mixed effect models (LME) and likelihood ratio tests were used to assess the impact of harmonic reduction on enjoyment. Results: NH listeners without simulation rated segments with the first four harmonics (F3+F2+F1+F0) most pleasant and natural (p 

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A Comparison of Surgical Treatments for Superior Semicircular Canal Dehiscence: A Systematic Review

Objective: We investigate the postoperative subjective and objective outcomes of different surgical treatments for superior semicircular canal dehiscence (SSCD): vestibular signs, auditory signs, vestibular evoked myogenic potential test, pure tone audiogram, speech audiogram, or video-nystagmography. Data Sources: An electronic search performed in the PubMed, Cochrane Library, and EMBASE databases on 15th of September 2015. A systematic search was conducted. Articles were included if written in English, Dutch, German, or French language. Study Selection: Original studies reporting on the pre and postoperative subjective and/or objective outcomes of surgical treatments for superior semicircular canal dehiscence were included. Data Extraction: The methodological quality of the studies was independently assessed by two reviewers using a constructed critical appraisal, to assess the directness of evidence and the risk of bias. The results of the pre and postoperative subjective and/or objective outcomes were extracted. Data Synthesis: Comparative study was conducted. Conclusion: Surgical treatment for SSCD is particularly effective for vestibular symptoms and there is no evidence for improvement of hearing loss after surgical treatment. Since plugging using transmastoid approach had a lower complication rate, lower revision rate, and a shorter hospital stay, this treatment is recommended in high disabled SSCD patients.

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X-linked Malformation and Cochlear Implantation

Objective: To evaluate if cochlear implantation is safe and constitutes an option for hearing rehabilitation of children with x-linked inner ear malformation. Study Design: Retrospective patient review in combination with a multidisciplinary follow-up. Setting: Tertiary referral hospital and cochlear implant program. Patients: Ten children with severe-profound mixed hearing loss and radiological findings consistent with Incomplete Partition type 3 cochlear malformation received cochlear implants during the years 2007 to 2015. Nine of the children had a mutation affecting the gene POU3F4 on Xq21. Intervention: Cochlear implantation. Main Outcome Measures: Surgical events, intraoperative measures and electrical stimulation levels, hearing and spoken language abilities. Results: In all, 15 cochlear implantations were performed. In three cases the electrode was found to be in the internal auditory canal on intraoperative x-ray and repositioned successfully. One child had a postoperative rhinorrhea confirmed to be cerebrospinal fluid but this resolved on conservative treatment. No severe complications occurred. Postoperative electrical stimulation levels were higher in 9 of 10 children, as compared with typically reported average levels in patients with a normal cochlea. Eight patients developed spoken language to various degrees while two were still at precommunication level. However, speech recognition scores were lower than average pediatric cases. Conclusion: Cochlear implantation is a safe procedure for children with severe-profound mixed hearing loss related to POU3F4 mutation inner ear malformation. The children develop hearing and spoken language but outcome is below average for pediatric CI recipients.

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Tinnitus Suppression After Auditory Brainstem Implantation in Patients With Neurofibromatosis Type-2

Objective: To evaluate whether an auditory brainstem implant (ABI) can impact levels of tinnitus in neurofibromatosis type-2 (NF2) patients who have undergone translabyrinthine craniotomy for vestibular schwannoma (VS) removal and to evaluate the burden of tinnitus in these patients. Study Design: A retrospective case series and patient survey. Setting: Tertiary neurotologic referral center. Patients: NF2 patients who underwent translabyrinthine removal of VS and ABI placement between 1994 and 2015. Interventions: A survey, retrospective review and two validated tinnitus handicap questionnaires (tinnitus handicap inventory [THI] and tinnitus visual analogue scale [VAS]) were used to characterize the degree of tinnitus in NF2 patients and whether an ABI can alter tinnitus levels. Main Outcome Measures(s): Survey results, THI and VAS scores. Results: One hundred twelve ABI users were contacted and 43 patients (38.3)% responded to our survey. Tinnitus was reported in 83.7% of patients. The THI score for responders was 17.8 ± 20.5 standard deviation (SD). For survey participants, the ABI reduced tinnitus levels (mean VAS: Off = 3.5; On 1-h = 2.1; p = 0.048). For patients who subjectively reported that the ABI reduced tinnitus loudness, tinnitus levels were immediately reduced on ABI activation and after 1 hour of use (mean VAS: Off = 4.8; On = 2.4; On 1-h = 1.8; p 

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The Effect of Scala Tympani Morphology on Basilar Membrane Contact With a Straight Electrode Array: A Human Temporal Bone Study

Hypothesis: Scala tympani morphology influences the insertion dynamics and intra-scalar position of straight electrode arrays. Background: Hearing preservation is the goal of cochlear implantation with current thin straight electrode arrays. These hug the lateral wall, facilitating full, atraumatic insertions. However, most studies still report some postoperative hearing loss. This study explores the influence of scala tympani morphology on array position relative to the basilar membrane and its possible contribution to postoperative hearing loss. Materials and Methods: Twenty-six fresh-frozen human temporal bones implanted with a straight electrode array were three-dimensionally reconstructed from micro-photographic histological sections. Insertion depth and the proximity between the array and basilar membrane were recorded. Lateral wall shape was quantified as a curvature ratio. Results: Insertion depths ranged from 233 to 470 degrees. The mean first point of contact between the array and basilar membrane was 185 degrees; arrays tended to remain in contact with the membrane after first contacting it. Eighty-nine and 93% of arrays that reached the upper basal (>240–360 degrees) and second (>360–720 degrees) turns respectively contacted the basilar membrane in these regions. Scalar wall curvature ratio decreased significantly (the wall became steeper) from the basal to second turns. This shift correlated with a reduced distance between the array and basilar membrane. Conclusion: Scala tympani morphology influences the insertion dynamics and intra-scalar position of a straight electrode array. In addition to gross trauma of cochlear structures, contact between the array and basilar membrane and how this impacts membrane function should be considered in hearing preservation cases.

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Inhibiting p21-Activated Kinase Induces Cell Death in Vestibular Schwannoma and Meningioma via Mitotic Catastrophe

Hypothesis: p21-activated kinase (PAK) regulates signaling pathways that promote cell survival and proliferation; therefore, pharmacological inhibition of PAK will induce cell death in vestibular schwannomas (VS) and meningiomas. Background: All VS and many meningiomas result from loss of the neurofibromatosis type 2 (NF2) gene product merlin, with ensuing PAK hyperactivation and increased cell proliferation/survival. Methods: The novel small molecule PAK inhibitors PI-8 and PI-15—tested in schwannoma and meningioma cells—perturb molecular signaling and induce cell death. 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide, flow cytometry, and terminal deoxynucleotidyl transferase dUTP nick end labeling assay analyzed PAK inhibitors’ effect on cell viability, cell cycle, and cell death, respectively. Western blots evaluated activation and expression of cell proliferation, apoptotic, and mitotic catastrophe markers. Light microscopy evaluated cell morphology, and immunocytochemistry analyzed cellular localization of phospho-Merlin and autophagy-related protein. Results: Treatment with PI-8 and PI-15 decreased cell viability at 0.65 to 3.7 μM 50% inhibitory concentration (IC50) in schwannoma and meningioma cells. Terminal deoxynucleotidyl transferase dUTP nick end labeling and immunocytochemistry studies show that PI-8 and PI-15 induce mitotic catastrophe but not apoptosis in HEI193 cells while in BenMen1 cells, PI-8 induces autophagy and mitotic catastrophe. PI-15 induces apoptosis in BenMen1 cells. PAK inhibitor treated cells show phospho-Merlin localized to over-duplicated centrosomes of dividing cells, multiple enlarged nuclei, and misaligned/missegregated chromosomes—markers for mitotic catastrophe. Increased autophagy-related protein levels in the nucleus confirmed this cell death type. PI-8 and PI-15 inhibits PAK in both cell lines. However, only PI-15 inhibits v-akt murine thymoma viral oncogene homolog in BenMen1 cells. Conclusion: PAK inhibitors induce cell death in schwannoma and meningioma cells, at least in part, by mitotic catastrophe.

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Cochlear Implantation in the Elderly: Does Age Matter?

Objective: To compare the outcome of hearing rehabilitation in younger versus older adult cochlear implant recipients. Analysis of surgical and postoperative complications, as well as the number of auditory therapy sessions in the two age groups. Study Design: Individual retrospective cohort study. Methods: A cohort of 145 postlingually deafened adults was evaluated in this study. The patients were divided into two age groups based on the age at implantation: Group I, 18 to 69 years; and Group II, 70 and older. Postoperative hearing performance was measured based on the German Freiburg monosyllabic word test (FM) and the Oldenburg sentence test (OLSA). Results: Postoperative hearing evaluation results in both groups plateaued and remained constant after 12 months of implantation. The results remained constant at the 2 and 3-year time intervals. There was a significant difference in complications arising after cochlear implantation. Group II showed more cases of vertigo and dysgeusia. The number of auditory therapy sessions in both groups was similar. Conclusion: Cochlear implantation in the elderly is highly effective; the postoperative hearing performance is at the same level as younger adult recipients. Complex hearing tasks, such as hearing in background noise, requires an equally long time for comprehension. The recovery period of vestibular dysfunction after surgery may be longer in the elderly. Auditory therapy rehabilitation is not more time consuming in the elderly compared with the younger counterparts.

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Response to Comment on “A Systematic Review on Complications of Tissue Preservation Surgical Techniques in Percutaneous Bone Conduction Hearing Devices”

No abstract available

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Usefulness of the Saccharin Test for Assessment of Eustachian Tube Function in Patients With Chronic Otitis Media With Perforation

Objective: The eustachian tube (ET) has two important functions, ventilation and clearance of the middle ear. We evaluated the ET ventilator function by the sonotubometry and the inflation-deflation test that are widely used today, and the ET clearance function by the saccharin test. Results of both tests were compared with surgical outcomes, and assessed which test was more closely related to the surgical outcomes. Study Design: Prospective case series. Setting: A single university hospital. Patients and Intervention: One hundred four ears of 95 patients with chronic otitis media with perforation underwent type I tympanoplasty. Main Outcomes and Measures: Based on sonotubometry and the inflation–deflation test, the patients were divided into normal, stenosis, and patulous types. Based on the saccharin test, they were divided into a normal function group and a partial/gross dysfunction group. Surgical outcomes about the hearing results and the condition of the eardrum were assessed 1 year postoperatively. Preoperative evaluation of ET function was compared with the surgical outcome. Results: Sonotubometry and inflation-deflation tests showed there was no significant difference among the groups about the hearing improvement and the surgical success rate. The saccharin test showed that the rate of the hearing improvement was significantly lower in the gross dysfunction group and that the success rate of Type I tympanoplasty was significantly higher in the normal group than in the dysfunction group. Conclusion: There is a relationship between the saccharin test results and surgical outcomes.

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Effect of Goggle Slippage on the Video Head Impulse Test Outcome and Its Mechanisms

Objectives: The aim of this study was to quantitatively measure the tightness of the goggle strap during the video head impulse test (vHIT) and to identify slippage-induced artifacts according to tightness. We aimed to elucidate the mechanism of faulty gain caused by goggle slippage and explain the typical artifacts associated with it. Subjects and Methods: An endotracheal tube cuff manometer was coupled to the EyeSeeCam vHIT system (Interacoustics, Assens, Denmark) to monitor strap tightness. The instantaneous gain (40, 60, and 80 ms) and regression gain were compared in eight healthy subjects under the following strap tightness conditions: loose (25 cm H2O), tight (35 cm H2O), and very tight (45 cm H2O). To elucidate the mechanism of faulty gain caused by goggle slippage, a fake fixed pupil with a vestibule ocular reflex (VOR) gain of 0 was attached to the subject's eyelid. The faulty gain recording pattern was analyzed as the tightness of the strap was decreased. Results: The most common slippage-induced artifacts were: 1) initial backward eye movement toward the head movement, 2) acceleration bumps, 3) high gain, and 4) deceleration bumps. At 40 ms, the gain was significantly lower in the 25 cm H2O condition (0.68 ± 0.32 cm H2O) compared with the 45 cm H2O condition (0.90 ± 0.26 cm H2O). At 80 ms, the gain was higher for the 25 cm H2O condition (1.24 ± 0.27 cm H2O) compared with the 45 cm H2O condition (1.16 ± 0.30 cm H2O). These findings were progressively more obvious as the tightness of the strap decreased in a dose-dependent manner. When the fake pupil was recorded, initial backward eye movement toward the head movement (negative VOR gain) and eye tracing mimicking a small VOR (positive VOR gain) were recorded, despite the fake pupil having absolutely no movement. These artifact recordings are presumed to be related to the faulty low (40 ms) and high (80 ms) gain calculation. Conclusions: Slippage-induced artifacts are presumed to be because of the slingshot-like movement of the goggles during head movement in three different phases (lagging, overshooting, and bouncing of the goggles). Monitoring the pressure of the strap tightness may be a solution for minimizing this slippage. A strap tightness of at least 45 cm H2O is required for reliable vHIT recording and gain calculations.

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Posterior Fossa Spontaneous Cerebrospinal Fluid Leaks

Objective: Describe the diagnosis and management of spontaneous lateral skull base cerebrospinal fluid (CSF) leaks that originate from the posterior fossa. Study Design: Retrospective case review. Setting: Tertiary university hospital. Patients: Adult patients from 2005 to 2015 who underwent surgical repair of a spontaneous lateral skull base CSF leak with intraoperative confirmation of a posterior fossa leak source. Intervention: Surgical repair. Main Outcome Measures: CSF leak resolution. Results: Five patients had CSF leaks from the posterior fossa. The mean age at presentation was 54 years old (range, 19–79), the mean body mass index (BMI) was 32.6 (standard deviation [SD], 8.4), and the mean follow-up length was 34.6 months (SD, 19.4). Presentations did not differ from CSF leaks through middle fossa defects, including three patients with a history of meningitis and all patients with clear otorrhea following tympanostomy tube placement. All patients had resolution of the leak after surgical repair, but two patients required revision surgery for persistent leaks and one patient had a postoperative infection. Surgical approaches included one middle fossa, two transmastoid, one combined middle fossa/transmastoid, and one transcanal. Radiographic studies suggested a posterior fossa source in all cases but findings were often subtle. Conclusion: Posterior fossa CSF leaks represent a rare subset of spontaneous lateral skull base leaks. Diligent radiographic review and intraoperative assessment of the posterior fossa plate are crucial. The size and location of the defect dictates the optimal surgical approach. Surgeons should consider a posterior fossa source in failed repairs or when the initial surgery did not fully evaluate the posterior fossa plate.

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The First Reported Case of Recurrent Carcinoid Tumor in the External Auditory Canal

Objective: Cutaneous neuroendocrine lesions of the external auditory canal (EAC) are exceptionally rare, with only five cases reported in the literature. In this case report, we present a patient with a recurrent carcinoid tumor in the EAC, which has yet to be described. Patients: A 38-year-old woman presenting with otalgia, aural fullness, and decreased hearing was found to have a recurrent EAC carcinoid tumor, 8-years after initial resection at an outside facility. Interventions: The recurrent tumor involved much of the proximal, anterior bony ear canal and was trans-tympanic, extending to the middle ear and epitympanum; therefore, a lateral temporal bone resection was performed to ensure complete resection. Main Outcome Measures: Surgical pathology confirmed the presence a recurrent carcinoid tumor in the EAC, with immunohistochemistry positive for pancytokeratin (MAK6), CD56, and synaptophysin, with chromogranin showing rare cells positive for cytoplasmic granules. There was no evidence of metastasis. Results: Lateral temporal bone resection was successful and the patient is being followed with annual imaging. The patient is considering future hearing rehabilitation with a bone anchored hearing device. Conclusion: This case report highlights the first known case of recurrent carcinoid tumor in the EAC, treated with lateral temporal bone resection. Clinical presentation, imaging, treatment, and pathology are reviewed along with a review of the literature.

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Otologic and Audiologic Outcomes in Pediatric Patients With Velo-Cardio-Facial (22q11 Deletion) Syndrome

Objective: The focus of this study was to evaluate the prevalence, type, and severity of hearing impairment in patients with velo-cardio-facial syndrome (VCFS) and to compare these characteristics with patient demographics and other otologic factors. Study Design: Retrospective analysis of the AudGen Database. Setting: Tertiary academic referral center. Patients: Pediatric patients in AudGenDB with a diagnosis of velo-cardio-facial syndrome or DiGeorge syndrome. Interventions: Appropriate audiologic, otologic, and demographic data were recorded. Main Outcome Measure: Four hundred six patients met inclusion criteria. Data for each patient were selected based on their encounters with earliest complete audiometric data, and hearing loss (HL) was defined as greater than 15 dB HL at any threshold by pure tone or greater than 20 dB HL by soundfield audiometry. The patients were then stratified by type and severity of HL, and available otologic and medical conditions were documented. Results: Two hundred forty eight (60.7%) patients had HL. Of the 391 individual HL ears, 127 had conductive, 22 had sensorineural, 115 had mixed, and 127 had unspecified hearing loss. 57% of the loss was bilateral. Patients with mixed HL had significantly worse pure-tone averages (PTAs). Conclusion: HL is prevalent in patients with VCFS. The hearing loss is primarily of conductive origin, with a predisposition to be bilateral, with mixed HL typically have more severe loss. There were a large number of patients with sensorineural hearing loss components as well. Further studies are needed to investigate the causal nature of the hearing impairment in VCFS, to better aid otolaryngologist and audiologists when assessing these patients.

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The Natural History of Petroclival Meningiomas: A Volumetric Study

Objectives: This study characterizes primary petroclival meningioma growth rates, before intervention, using volumetric analysis. In addition, predictors of growth are analyzed. Methods: Patients with previously untreated petroclival meningiomas were retrospectively reviewed (1999–2015). Image analysis software was used to perform volumetric analyses of tumor size and growth. Three-dimensional segmentation volumetric analyses were compared with volumes estimated utilizing three orthogonal dimensions. Tumor growth was defined as a 15% increase in volume. Results: Thirty-four patients who underwent at least two magnetic resonance imaging (MRI) studies before intervention were included. The mean age was 55.2 years, and 65.7% were women. The mean tumor volume at presentation was 5.6 cm3 (range, 0.1–25.8 cm3) as determined from segmentation volumetric analysis. At a mean follow-up of 44.5 months (range, 3.7–125.1 mo), 88.2% of tumors grew. The mean annual volumetric growth rate was 2.38 cm3/yr (−0.63 to 25.9 cm3/yr). Tumor volume, T2 hyperintensity within the tumor, peritumoral edema, and ataxia and/or cerebellar symptoms at presentation were all significantly associated with greater rates of tumor growth. Ultimately, 10 (29.4%) patients underwent treatment during the follow-up period. Conclusion: Our experience demonstrates that the vast majority (88%) of untreated petroclival meningiomas grow; the mean volumetric growth rate was noted to be 2.38 cm3/yr. We found a significant association between increased growth rate and larger tumor size at diagnosis, T2 hyperintensity within the tumor, peritumoral edema, and the presence of ataxia and/or cerebellar symptoms.

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Cochleariform Process Abutment on TBCT in Early Congenital Cholesteatoma

Objectives: To study the influence of the cochleariform process abutment (CPA) of early congenital cholesteatomas (CC) (ECCs) on surgical outcomes. Study Design: Retrospective case review. Setting: University hospital otology referral clinic. Patients: Two hundred consecutive pediatric ECC patients. Interventions: The patients were classified into three groups based on the temporal bone computed tomography (TBCT) findings of ECC: A) the absence of CPA, B) the presence of CPA, and C) the presence of CPA and extension posterior to the malleus handle. Main Outcome Measures: Residual CC according to each of the TBCT and surgical stages during 34.9 ± 20.1 months of follow-up. Results: Group A was significantly associated with effortless delivery without matrix tearing (77%) even with simple myringotomy. Only 17% of group B experienced this outcome, and troublesome matrix tearing occurred in significantly more patients (p 

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