Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines on Surgical Resection for the Treatment of Patients With Vestibular Schwannomas.

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines on Surgical Resection for the Treatment of Patients With Vestibular Schwannomas.

Neurosurgery. 2017 Dec 20;:

Authors: Hadjipanayis CG, Carlson ML, Link MJ, Rayan TA, Parish J, Atkins T, Asher AL, Dunn IF, Corrales CE, Van Gompel JJ, Sughrue M, Olson JJ

Abstract
QUESTION 1: What surgical approaches for vestibular schwannomas (VS) are best for complete resection and facial nerve (FN) preservation when serviceable hearing is present?
RECOMMENDATION: There is insufficient evidence to support the superiority of either the middle fossa (MF) or the retrosigmoid (RS) approach for complete VS resection and FN preservation when serviceable hearing is present.
QUESTION 2: Which surgical approach (RS or translabyrinthine [TL]) for VS is best for complete resection and FN preservation when serviceable hearing is not present?
RECOMMENDATION: There is insufficient evidence to support the superiority of either the RS or the TL approach for complete VS resection and FN preservation when serviceable hearing is not present.
QUESTION 3: Does VS size matter for facial and vestibulocochlear nerve preservation with surgical resection?
RECOMMENDATION: Level 3: Patients with larger VS tumor size should be counseled about the greater than average risk of loss of serviceable hearing.
QUESTION 4: Should small intracanalicular tumors (<1.5 cm) be surgically resected?
RECOMMENDATION: There are insufficient data to support a firm recommendation that surgery be the primary treatment for this subclass of VSs.
QUESTION 5: Is hearing preservation routinely possible with VS surgical resection when serviceable hearing is present?
RECOMMENDATION: Level 3: Hearing preservation surgery via the MF or the RS approach may be attempted in patients with small tumor size (<1.5 cm) and good preoperative hearing.
QUESTION 6: When should surgical resection be the initial treatment in patients with neurofibromatosis type 2 (NF2)?
RECOMMENDATION: There is insufficient evidence that surgical resection should be the initial treatment in patients with NF2.
QUESTION 7: Does a multidisciplinary team, consisting of neurosurgery and neurotology, provides the best outcomes of complete resection and facial/vestibulocochlear nerve preservation for patients undergoing resection of VSs?
RECOMMENDATION: There is insufficient evidence to support stating that a multidisciplinary team, usually consisting of a neurosurgeon and a neurotologist, provides superior outcomes compared to either subspecialist working alone.
QUESTION 8: Does a subtotal surgical resection of a VS followed by stereotactic radiosurgery (SRS) to the residual tumor provide comparable hearing and FN preservation to patients who undergo a complete surgical resection?
RECOMMENDATION: There is insufficient evidence to support subtotal resection (STR) followed by SRS provides comparable hearing and FN preservation to patients who undergo a complete surgical resection.
QUESTION 9: Does surgical resection of VS treat preoperative balance problems more effectively than SRS?
RECOMMENDATION: There is insufficient evidence to support either surgical resection or SRS for treatment of preoperative balance problems.
QUESTION 10: Does surgical resection of VS treat preoperative trigeminal neuralgia more effectively than SRS?
RECOMMENDATION: Level 3: Surgical resection of VSs may be used to better relieve symptoms of trigeminal neuralgia than SRS.
QUESTION 11: Is surgical resection of VSs more difficult (associated with higher facial neuropathies and STR rates) after initial treatment with SRS?
RECOMMENDATION: Level 3: If microsurgical resection is necessary after SRS, it is recommended that patients be counseled that there is an increased likelihood of a STR and decreased FN function.  The full guideline can be found at: http://ift.tt/2CVD7JB.

PMID: 29309632 [PubMed - as supplied by publisher]

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The Effect of Vestibular Rehabilitation Therapy Program on Sensory Organization of Deaf Children With Bilateral Vestibular Dysfunction.

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The Effect of Vestibular Rehabilitation Therapy Program on Sensory Organization of Deaf Children With Bilateral Vestibular Dysfunction.

Acta Med Iran. 2017 Nov;55(11):683-689

Authors: Ebrahimi AA, Jamshidi AA, Movallali G, Rahgozar M, Haghgoo HA

Abstract
The purpose of this study was to determine the effect of vestibular rehabilitation therapy program on the sensory organization of deaf children with bilateral vestibular dysfunction. This cross-sectional and analytic study was conducted on 24 students between the age of 7 and 12 years (6 girls and 18 boys) with the profound sensorineural hearing loss (PTA>90 dB). They were assessed through the balance subtest in Bruininks-Oseretsky test of motor proficiency (BOTMP). For children which the total score of the balance subtest was 3 standard deviation lower than their peers with typical development, vestibular function testing was completed pre-intervention. Posturography Sensory organization testing (SOT) was completed pre- and post-intervention with SPS (Synapsys, Marseille, France). Children with bilateral vestibular impairment were randomly assigned to either the exercise or control group. Exercise intervention consisted of compensatory training, emphasizing enhancement of visual and somatosensory function, and balance training. The exercise group entered in vestibular rehabilitation therapy program for 8 weeks. The children initially participating in the control group were provided the exercise intervention following the post-test. Based on the results there was significant difference in condition 5 and 6, areas of limits of stability (LOS), vestibular ratio and global score in posturography at the end of the intervention, but there was no significant difference in the control group in posturography (P<0.05). The results indicated that testing of vestibular, and postural control function, as well as intervention for deficiencies identified, should be included in deaf children rehabilitation program.

PMID: 29307157 [PubMed - in process]

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A C-terminal nonsense mutation links PTPRQ with autosomal-dominant hearing loss, DFNA73.

A C-terminal nonsense mutation links PTPRQ with autosomal-dominant hearing loss, DFNA73.

Genet Med. 2017 Oct 12;:

Authors: Eisenberger T, Di Donato N, Decker C, Delle Vedove A, Neuhaus C, Nürnberg G, Toliat M, Nürnberg P, Mürbe D, Bolz HJ

Abstract
PurposeHearing loss is genetically extremely heterogeneous, making it suitable for next-generation sequencing (NGS). We identified a four-generation family with nonsyndromic mild to severe hearing loss of the mid- to high frequencies and onset from early childhood to second decade in seven members.MethodsNGS of 66 deafness genes, Sanger sequencing, genome-wide linkage analysis, whole-exome sequencing (WES), semiquantitative reverse-transcriptase polymerase chain reaction.ResultsWe identified a heterozygous nonsense mutation, c.6881G>A (p.Trp2294*), in the last coding exon of PTPRQ. PTPRQ has been linked with recessive (DFNB84A), but not dominant deafness. NGS and Sanger sequencing of all exons (including alternatively spliced 5' and N-scan-predicted exons of a putative "extended" transcript) did not identify a second mutation. The highest logarithm of the odds score was in the PTPRQ-containing region on chromosome 12, and p.Trp2294* cosegregated with hearing loss. WES did not identify other cosegregating candidate variants from the mapped region. PTPRQ expression in patient fibroblasts indicated that the mutant allele escapes nonsense-mediated decay (NMD).ConclusionKnown PTPRQ mutations are recessive and do not affect the C-terminal exon. In contrast to recessive loss-of-function mutations, c.6881G>A transcripts may escape NMD. PTPRQTrp2294* protein would lack only six terminal residues and could exert a dominant-negative effect, a possible explanation for allelic deafness, DFNA73, clinically and genetically distinct from DFNB84A.GENETICS in MEDICINE advance online publication, 12 October 2017; doi:10.1038/gim.2017.155.

PMID: 29309402 [PubMed - as supplied by publisher]

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Identification of Novel PTPRQ and MYO1A Mutations in An Iranian Pedigree with Autosomal Recessive Hearing Loss.

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Identification of Novel PTPRQ and MYO1A Mutations in An Iranian Pedigree with Autosomal Recessive Hearing Loss.

Cell J. 2018 Apr;20(1):127-131

Authors: Talebi F, Ghanbari Mardasi F, Mohammadi Asl J, Tizno S, Najafvand Zadeh M

Abstract
Autosomal recessive non-syndromic hearing loss (ARNSHL) is defined as a genetically heterogeneous disorder. The aim of the present study was to screen for pathogenic variants in an Iranian pedigree with ARNSHL. Next-generation targeted sequencing of 127 deafness genes in the proband detected two novel variants, a homozygous missense variant in PTPRQ (c.2599 T>C, p.Ser867Pro and a heterozygous missense variant in MYO1A (c.2804 T>C, p.Ile935Thr), both of which were absent in unaffected sibs and two hundred unaffected controls. Our results suggest that the homozygous PTPRQ variant maybe the pathogenic variant for ARNSHL due to the recessive nature of the disorder. Nevertheless, the heterozygous MYO1A may also be involved in this disorder due to the multigenic pattern of ARNSHL. Our data extend the mutation spectrum of PTPRQ and MYO1A, and have important implications for genetic counseling in unaffected sibs of this family. In addition, PTPRQ and MYO1A pathogenic variants have not to date been reported in the Iranian population.

PMID: 29308629 [PubMed]

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Neural representation of octave illusion in the human cortex revealed with functional magnetic resonance imaging

Publication date: Available online 8 January 2018
Source:Hearing Research
Author(s): Keita Tanaka, Hiroki Kurasaki, Shinya Kuriki
The auditory “octave illusion” arises when dichotic tones, presented one octave apart, alternate rapidly between the ears. This study aimed to explore the link between the perception of illusory pitches and brain activity during presentation of dichotic tones. We conducted a behavioral study of how participants perceived binaural dichotic tones of octave illusions and classified them, based on the reported percepts, in an illusion (ILL) group, without an illusion (non-ILL) group, and others. We recorded brain activity using functional magnetic resonance imaging and analyzed the activation due to dichotic illusion tones. The activation in the bilateral planum polare in the auditory cortex was significantly larger in the ILL group than in the non-ILL group. In the right premotor cortex, the non-ILL group showed a significantly larger activation than did the ILL group, suggesting that the sensation of the meter to the stimulus sound was significant in the non-ILL but not in the ILL group. The results indicated that the activity in these areas was related to the occurrence of octave illusions. The nonsignificant sensation of the meter to the stimulus sound in the ILL group may be consistent with the perception of octave illusion.



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Neural representation of octave illusion in the human cortex revealed with functional magnetic resonance imaging

Publication date: Available online 8 January 2018
Source:Hearing Research
Author(s): Keita Tanaka, Hiroki Kurasaki, Shinya Kuriki
The auditory “octave illusion” arises when dichotic tones, presented one octave apart, alternate rapidly between the ears. This study aimed to explore the link between the perception of illusory pitches and brain activity during presentation of dichotic tones. We conducted a behavioral study of how participants perceived binaural dichotic tones of octave illusions and classified them, based on the reported percepts, in an illusion (ILL) group, without an illusion (non-ILL) group, and others. We recorded brain activity using functional magnetic resonance imaging and analyzed the activation due to dichotic illusion tones. The activation in the bilateral planum polare in the auditory cortex was significantly larger in the ILL group than in the non-ILL group. In the right premotor cortex, the non-ILL group showed a significantly larger activation than did the ILL group, suggesting that the sensation of the meter to the stimulus sound was significant in the non-ILL but not in the ILL group. The results indicated that the activity in these areas was related to the occurrence of octave illusions. The nonsignificant sensation of the meter to the stimulus sound in the ILL group may be consistent with the perception of octave illusion.



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Neural representation of octave illusion in the human cortex revealed with functional magnetic resonance imaging

Publication date: Available online 8 January 2018
Source:Hearing Research
Author(s): Keita Tanaka, Hiroki Kurasaki, Shinya Kuriki
The auditory “octave illusion” arises when dichotic tones, presented one octave apart, alternate rapidly between the ears. This study aimed to explore the link between the perception of illusory pitches and brain activity during presentation of dichotic tones. We conducted a behavioral study of how participants perceived binaural dichotic tones of octave illusions and classified them, based on the reported percepts, in an illusion (ILL) group, without an illusion (non-ILL) group, and others. We recorded brain activity using functional magnetic resonance imaging and analyzed the activation due to dichotic illusion tones. The activation in the bilateral planum polare in the auditory cortex was significantly larger in the ILL group than in the non-ILL group. In the right premotor cortex, the non-ILL group showed a significantly larger activation than did the ILL group, suggesting that the sensation of the meter to the stimulus sound was significant in the non-ILL but not in the ILL group. The results indicated that the activity in these areas was related to the occurrence of octave illusions. The nonsignificant sensation of the meter to the stimulus sound in the ILL group may be consistent with the perception of octave illusion.



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