MyMedR

Congenital bilateral facial nerve hypoplasia with sensorineural hearing loss: a case report

Hamizan AW, Yean KT, Abdullah A

Int J Pediatr Otorhinolaryngol, 2012 Mar;76(3):455-9.

PMID: 22281372 DOI: 10.1016/j.ijporl.2012.01.002

A 3-year-old child presented with congenital bilateral facial nerve palsy with bilateral profound sensorineural hearing loss. High Resolution Computed Tomogram (HRCT) of the temporal bones found bilateral atresia of cochlear nerve canals, incomplete partition of the cochleae and narrow facial nerve canals. Magnetic resonance imaging (MRI) revealed bilateral hypoplasia of facial nerves and aplasia of both vestibulocochlear nerves. There have been no other reported cases with this presentation. The possible aetiology and treatment options for the patient are discussed. We highlighted the review of aplasia/hypoplasia of the facial nerve and hypoplasia of cochlear nerve canal.

Matched MeSH terms: Hearing Loss, Sensorineural/congenital*

Fulltext Large vestibular aqueduct syndrome

Dipak S, Prepageran N, Sazila AS, Rahmat O, Raman R

Med J Malaysia, 2005 Oct;60(4):489-91.

PMID: 16570713

Large Vestibular Aqueduct Syndrome is a congenital malformation of the temporal bone characterised by early onset of sensorineural hearing loss and vestibular disturbance. Familial large vestibular aqueduct syndrome suggests autosomal recessive or X-linked inheritance and accounts for non-syndromic sensorineural hearing loss in these patients.

Matched MeSH terms: Hearing Loss, Sensorineural/congenital*

Fulltext Bilateral microtia, canal atresia and aplasia of cochleovestibular nerve

Asma A, Roslenda AR, Fadzilah I, Mazita A, Marina MB, Ab Aziz A

Med J Malaysia, 2017 04;72(2):135-137.

PMID: 28473682 MyJurnal

A six-month-old baby with congenital patent ductus arteriosus (PDA), bilateral microtia and canal atresia was referred for hearing assessment. The audiology assessment revealed bilateral profound hearing loss, which is atypical for a case of pure canal atresia. Imaging was performed much earlier than usual and, as suspected, the patient also had bilateral severe inner ear anomaly. It is extremely rare for a person to have both external and inner ear anomaly because of the different embryological origin. The only suitable hearing rehabilitation option for this kind of patients is brainstem implant. However, the parents had opted for sign language as a form of communication.

Matched MeSH terms: Hearing Loss, Sensorineural/congenital

Comparison of distortion product otoacoustic emission (DPOAE) and automated auditory brainstem response (AABR) for neonatal hearing screening in a hospital with high delivery rate

Ngui LX, Tang IP, Prepageran N, Lai ZW

Int J Pediatr Otorhinolaryngol, 2019 May;120:184-188.

PMID: 30844634 DOI: 10.1016/j.ijporl.2019.02.045

INTRODUCTION: Congenital hearing loss is one of the commonest congenital anomalies. Neonatal hearing screening aims to detect congenital hearing loss early and provide prompt intervention for better speech and language development. The two recommended methods for neonatal hearing screening are otoacoustic emission (OAE) and automated auditory brainstem response (AABR).

OBJECTIVE: To study the effectiveness of distortion product otoacoustic emission (DPOAE) and automated auditory brainstem response (AABR) as first screening tool among non-risk newborns in a hospital with high delivery rate.

METHOD: A total of 722 non-risk newborns (1444 ears) were screened with both DPOAE and AABR prior to discharge within one month. Babies who failed AABR were rescreened with AABR ± diagnostic auditory brainstem response tests within one month of age.

RESULTS: The pass rate for AABR (67.9%) was higher than DPOAE (50.1%). Both DPOAE and AABR pass rates improved significantly with increasing age (p-value<0.001). The highest pass rate for both DPOAE and AABR were between the age of 36-48 h, 73.1% and 84.2% respectively. The mean testing time for AABR (13.54 min ± 7.47) was significantly longer than DPOAE (3.52 min ± 1.87), with a p-value of <0.001.

CONCLUSIONS: OAE test is faster and easier than AABR, but with higher false positive rate. The most ideal hearing screening protocol should be tailored according to different centre.

Matched MeSH terms: Hearing Loss, Sensorineural/congenital

Internal auditory canal stenosis in congenital sensorineural hearing loss

Sakina MS, Goh BS, Abdullah A, Zulfiqar MA, Saim L

Int J Pediatr Otorhinolaryngol, 2006 Dec;70(12):2093-7.

PMID: 16996619

Sensorineural hearing loss (SNHL) due to internal auditory canal (IAC) stenosis with hypoplasia of the cochleovestibular nerve is a rare disorder. The diagnosis of the IAC stenosis requires both high resolution computed tomography scan (HRCT) and magnetic resonance imaging (MRI).

Matched MeSH terms: Hearing Loss, Sensorineural/congenital*

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