Case report
Case report: “Auditory neuropathy” in a newborn caused by a cerebellopontine angle arachnoid cyst

https://doi.org/10.1016/j.ijporl.2008.02.003Get rights and content

Summary

We present a 6-week-old girl, referred because of failed newborn hearing screening in the right ear. Click-evoked oto-acoustic emissions were present in both ears, auditory brainstem responses (ABR) were present in the left but totally absent in the right ear. A magnetic resonance imaging (MRI) study revealed a large arachnoid cyst in the right cerebellopontine angle (CPA) and a diagnosis of “auditory neuropathy/auditory dyssynchrony” was established. A microsurgical resection of the cyst wall and fenestration was performed by a retro sigmoid approach. This is the first case in the literature of auditory neuropathy (AN) in an infant caused by a cerebellopontine angle arachnoid cyst.

Section snippets

Case presentation

A 6-week-old girl was admitted to our department after a failed neonatal hearing screening at the right ear. Newborn hearing screening was performed by automated auditory brainstem responses (AABR) at the age of 1 month. She was the 2nd child in a family of non-consanguineous parents. Pregnancy and birth were uneventful. Familial history was negative for hearing loss.

Clinical examination of the head and neck were unremarkable. Micro-otoscopy revealed a normal tympanic membrane at both ears. An

Discussion

To our best knowledge, this is the first report in the literature of a congenital posterior fossa arachnoid cyst presenting as “ auditory neuropathy” in an infant referred after universal neonatal hearing screening. Zada et al. presented a large series of 42 children younger than 2 years of age who had undergone craniotomy for fenestration of an arachnoid cyst [1]. In this series, 5 (12%) were located in the posterior fossa and none presented with hearing loss.

Arachnoidal cysts are

Conclusions

Abnormal ABR responses with presence of click-evoked OAE's in an infant require a complete diagnostic work-up including imaging studies of the posterior fossa and brain to exclude underlying anatomic abnormalities. This approach may not only have important diagnostic but also therapeutic consequences as illustrated in this case where a posterior fossa arachnoid cyst was the underlying pathology in an infant with a unilateral refer following neonatal hearing screening.

References (18)

There are more references available in the full text version of this article.

Cited by (0)

View full text