Retrocochlear hearing loss in infants: A case study of juvenile pilocytic astrocytoma

Abstract

Objective: The purpose of this paper was to describe a child with brainstem juvenile pilocytic astrocytoma (JPA) and associated auditory symptoms. Design: Case study of a 5-year-old female who presented with a complaint of headaches, accompanied with a history of symptoms that could be associated with a left unilateral congenital auditory neuropathy. She was later diagnosed with a large, left-sided posterior fossa mass determined to be a JPA. Study sample: Case study of a child identified at birth with a constellation of symptoms consistent with unilateral auditory neuropathy spectrum disorder (ANSD). Results: The early onset symptoms, that can be the same for ANSD or for a brainstem tumor that involves the auditory nerve, suggests that the JPA was congenital. This child, if initially followed by neurology may not have been identified earlier with this tumor, however a baseline MRI or monitoring neuroimaging may have been available for reference or led to earlier detection of the lesion. It may not have changed her management, however, this case stresses the importance of the recommendations in the ‘Guidelines for Identification and Management of Infants and Young Children with Auditory Neuropathy Spectrum Disorder’ that include a referral to a neurologist for a baseline assessment and neuroimaging. Conclusion: An undetected JPA can have the same audiological clinical presentation as a unilateral ANSD. This case description highlights the need to heighten awareness for neurological and radiological monitoring in cases of ANSD, especially when the presentation is unilateral.

Key Words::

• Auditory neuropathy
• auditory neuropathy spectrum disorder
• infant brain tumors
• juvenile pilocytic astrocytoma
• unilateral
• retrocochlear
• infants
• newborn hearing screening

Acknowledgements

A preliminary version of this work was presented at the International Conference on Newborn Hearing Screening, Cernobbio (Como Lake), Italy, June 9, 2010. Appreciation and gratitude are expressed to Dr. Anne Tharpe and the Audiology Department at the Children's Hospital and Research Center at Oakland. In addition, the author would like to thank the three anonymous reviewers for their comments to improve the paper.

Declaration of interest: The author reports no conflicts of interest.