Vestibular nerve deficiency and vestibular function in children with unilateral hearing loss caused by cochlear nerve deficiency

AbstractUnilateral sensorineural hearing loss is a common symptom of vestibular schwannomas in adolescent patients with neurofibromatosis type 2 or sporadic vestibular schwannomas and is often the initial clinical feature. While rare cases of sensorineural impairment presenting as vision or hearing loss due to metastatic medulloblastoma are known, hearing loss as an isolated presenting symptom of primary malignant neuroepithelial tumors of the central nervous system has not been reported in the pediatric population so far. We present two adolescents with unilateral hearing loss due to cochlear nerve dysfunction as the only symptom of a primary nonmetastatic medulloblastoma of the WNT signaling pathway family members subgroup.

Download Full-text

Bony cochlear nerve canal stenosis and speech discrimination in pediatric unilateral hearing loss

The Laryngoscope ◽

10.1002/lary.25087 ◽

2015 ◽

Vol 125 (7) ◽

pp. 1691-1696 ◽

Cited By ~ 11

Author(s):

Patricia L. Purcell ◽

Ayaka J. Iwata ◽

Grace S. Phillips ◽

Angelisa M. Paladin ◽

Kathleen C. Y. Sie ◽

...

Keyword(s):

Hearing Loss ◽

Cochlear Nerve ◽

Unilateral Hearing Loss ◽

Speech Discrimination ◽

Canal Stenosis

Download Full-text

Hearing Loss in Pediatric Septo-Optic Dysplasia

Annals of Otology Rhinology & Laryngology ◽

10.1177/0003489419832629 ◽

2019 ◽

Vol 128 (6) ◽

pp. 485-489

Author(s):

Brian William Herrmann ◽

Campbell R. Hathaway ◽

Michael Fadell

Keyword(s):

Hearing Loss ◽

Early Identification ◽

Relevant Literature ◽

Population Based ◽

Cochlear Nerve ◽

Tympanostomy Tubes ◽

Predominant Form ◽

Children With Hearing Loss ◽

Cochlear Nerve Deficiency ◽

Hospital Clinic

Objective: To identify and characterize hearing loss (HL) in children with septo-optic dysplasia (SOD). Methods: Otologic and audiometric data for patients less than 18 years of age identified as having SOD who were seen in the Children’s Healthcare of Atlanta-Scottish Rite Hospital clinic between 2013 and 2017 were collected and reviewed through a HIPAA-compliant medical record search. Relevant literature was also reviewed with the assistance of Medline. Results: Sixty-four patients with SOD were identified, and 7 of those patients (10.9%) were diagnosed with hearing loss. Type of hearing loss was sensorineural (SNHL) in 5 patients (63%), mixed (MHL) in 1(14%), and conductive (CHL) in 1(14%). Bilateral loss presented in 60% (3/5) of SNHL patients, while the rest demonstrated unilateral loss. Unilateral findings included cochlear nerve deficiency (1) and atresia/microtia (1). Tympanostomy tubes were required in 57% (4/7) of SOD children with hearing loss. Amplification was successfully implemented in 86% (6/7). Conclusions: Hearing loss was found in nearly 11% of SOD children, and SNHL was identified as (63%) the predominant form of loss. To our knowledge, this is the first retrospective review of hearing loss in a pediatric SOD cohort and the first to report of cochlear nerve deficiency and atresia/microtia in this population. Based on these findings, early identification of hearing loss with imaging when appropriate and treatment of otitis in this population is recommended.

Download Full-text

A Case of Cochlear Nerve Deficiency Without Profound Sensorineural Hearing Loss

Otology & Neurotology ◽

10.1097/mao.0b013e318210b88c ◽

2011 ◽

Vol 32 (4) ◽

pp. 529-532 ◽

Cited By ~ 8

Author(s):

Ikuyo Miyanohara ◽

Keiichi Miyashita ◽

Koji Takumi ◽

Masayuki Nakajo ◽

Yuichi Kurono

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Cochlear Nerve ◽

Sensorineural Hearing ◽

Cochlear Nerve Deficiency

Download Full-text

Cochlear Implant Outcomes for Children With Auditory Neuropathy Spectrum Disorder

Perspectives on Hearing and Hearing Disorders in Childhood ◽

10.1044/hhdc19.2.75 ◽

2009 ◽

Vol 19 (2) ◽

pp. 75-84 ◽

Cited By ~ 6

Author(s):

Jaime Leigh ◽

Gary Rance ◽

Shani Dettman ◽

Richard Dowell

Keyword(s):

Hearing Loss ◽

Speech Perception ◽

Cochlear Implant ◽

Cochlear Implants ◽

Auditory Neuropathy ◽

Cochlear Nerve ◽

Spectrum Disorder ◽

Language Outcomes ◽

Outcomes For Children ◽

Cochlear Nerve Deficiency

Abstract Cochlear implantation is currently the intervention option of choice for many children with auditory neuropathy spectrum disorder (ANSD) who are unable to obtain benefit from conventional amplification. The aim of this study was to review the speech perception and language outcomes for children with ANSD who had received a cochlear implant and highlight specific clinical considerations for working with this population of children with hearing impairment who are being considered for implantation. Finds for the group of 17 children with ANSD using cochlear implants were compared to previously reported outcomes for children with sensori-neural (SN) type hearing loss using cochlear implants. Two children, identified with cochlear nerve deficiency pre-operatively, received no useful auditory percepts from their cochlear implant and discontinued device use. The remaining children demonstrated speech perception and language outcomes comparable to those observed for SN hearing loss peers using cochlear implants. This paper highlights a number of considerations for clinicians to be aware of and the importance of careful counseling pre-operatively regarding the potential for less-than-optimal outcomes, particularly for those children identified with cochlear nerve deficiency.

Download Full-text

Translabyrinthine nerve section: effect on tinnitus

The Journal of Laryngology & Otology ◽

10.1017/s1755146300090648 ◽

1984 ◽

Vol 98 (S9) ◽

pp. 287-293 ◽

Cited By ~ 15

Author(s):

David M. Barrs ◽

Derald E. Brackmann

Keyword(s):

Hearing Loss ◽

No Value ◽

Vestibular Nerve ◽

Cochlear Nerve ◽

Nerve Section ◽

Number Of Patients ◽

Better Than

AbstractThe effect on tinnitus in 110 patients undergoing translabyrinthine vestibular nerve section is presented. The effect on tinnitus of vestibular and cochlear nerve section (VCNS) together and vestibular nerve section (VNS) alone was studied. The number of patients improved with VCNS (61 per cent) was slightly better than with VNS alone (49 per cent). VNS alone worsened tinnitus more often than VCNS. Age, side involved, sex, etiology of vertigo, severity of tinnitus, interference with sleep and activities, slope of the pre-operative audiogram, and magnitude of the hearing loss had no value in predicting the change in tinnitus following either VNS or VCNS.

Download Full-text

Progression of Unilateral Hearing Loss in Children With and Without Ipsilateral Cochlear Nerve Canal Stenosis

Otology & Neurotology ◽

10.1097/mao.0000000000001452 ◽

2017 ◽

Vol 38 (6) ◽

pp. e138-e144 ◽

Cited By ~ 5

Author(s):

Patricia L. Purcell ◽

Justin R. Shinn ◽

Scott S. Coggeshall ◽

Grace Phillips ◽

Angelisa Paladin ◽

...

Keyword(s):

Hearing Loss ◽

Cochlear Nerve ◽

Unilateral Hearing Loss ◽

Canal Stenosis

Download Full-text

Introduction

The Journal of Laryngology & Otology ◽

10.1017/s1755146300090636 ◽

1984 ◽

Vol 98 (S9) ◽

pp. 287-287 ◽

Cited By ~ 1

Author(s):

D. Brackmann

Keyword(s):

Hearing Loss ◽

No Value ◽

Vestibular Nerve ◽

Cochlear Nerve ◽

Nerve Section ◽

Number Of Patients ◽

Better Than

AbstractThe effect on tinnitus in 110 patients undergoing translabyrinthine vestibular nerve section is presented. The effect on tinnitus of vestibular and cochlear nerve section (VCNS) together and vestibular nerve section (VNS) alone was studied. The number of patients improved with VCNS (61 per cent) was slightly better than with VNS alone (49 per cent). VNS alone worsened tinnitus more often than VCNS. Age, side involved, sex, etiology of vertigo, severity of tinnitus, interference with sleep and activities, slope of the pre-operative audiogram, and magnitude of the hearing loss had no value in predicting the change in tinnitus following either VNS or VCNS.

Download Full-text

Cochlear implantation after selective vestibular nerve section

The Journal of Laryngology & Otology ◽

10.1017/s002221511200326x ◽

2013 ◽

Vol 127 (3) ◽

pp. 311-313 ◽

Cited By ~ 3

Author(s):

C Martens ◽

A Csillag ◽

M Davies ◽

P Fagan

Keyword(s):

Magnetic Resonance Imaging ◽

Hearing Loss ◽

Case Report ◽

Magnetic Resonance ◽

Cochlear Implantation ◽

World Literature ◽

Vestibular Nerve ◽

Cochlear Nerve ◽

Resonance Imaging ◽

Nerve Section

AbstractIntroduction:Vestibular nerve section is a highly effective procedure for the control of vertigo in patients with Ménière's disease. However, hearing loss is a possible complication. If hearing loss occurs after vestibular nerve section, magnetic resonance imaging should make it possible to establish the presence or absence of an intact cochlear nerve.Method:Case report and review of the world literature concerning cochlear implantation after vestibular nerve section.Case report:We present a patient who developed subtotal hearing loss after vestibular nerve section. Magnetic resonance imaging was used to verify the presence of an intact cochlear nerve, enabling successful cochlear implantation.Conclusion:To our knowledge, this is the first reported case of cochlear implantation carried out after selective vestibular nerve section. Given recent advances in cochlear implantation, this case indicates that it is essential to make every effort to spare the cochlear nerve if vestibular nerve section is required. If hearing loss occurs after vestibular nerve section, magnetic resonance imaging should be undertaken to establish whether the cochlear nerve is intact.

Download Full-text