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Retrocochlear hearing loss in infants: a case study of juvenile pilocytic astrocytoma
1Children's Hospital & Research Center Oakland, Oakland, CA, USA. anachman@mail.cho.org
Insights
Juvenile pilocytic astrocytoma (JPA) in the brainstem can mimic auditory neuropathy spectrum disorder (ANSD). Early neurological and imaging assessments are crucial for diagnosing brainstem tumors in children with unilateral auditory symptoms.
Area of Science:
- Pediatric Neurology
- Neuro-oncology
- Audiology
Background:
- Juvenile pilocytic astrocytoma (JPA) is a common pediatric brain tumor.
- Auditory neuropathy spectrum disorder (ANSD) affects hearing despite normal otoacoustic emissions.
Observation:
- A 5-year-old female presented with headaches and symptoms consistent with unilateral auditory neuropathy spectrum disorder (ANSD).
- She was diagnosed with a left-sided posterior fossa juvenile pilocytic astrocytoma (JPA).
Findings:
- The patient's early-onset symptoms mimicked congenital ANSD, suggesting the JPA was congenital.
- Delayed diagnosis of the JPA highlights potential diagnostic challenges when symptoms overlap.
Implications:
- This case underscores the importance of neurological and radiological monitoring in children with unilateral ANSD.
- Guidelines recommending baseline assessment and neuroimaging for ANSD are critical for early detection of underlying conditions like JPA.
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.
