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Published on: February 29, 2020
Pediatric Idiopathic Intracranial Hypertension Presenting With Sensorineural Hearing Loss
Sietze Reitsma1, Robert Stokroos2, Jacobiene W Weber3
1Department of Otorhinolaryngology and Head and Neck Surgery, Maastricht University Medical Center+, Maastricht, The Netherlands sietze.reitsma@mumc.nl.
Insights
Idiopathic intracranial hypertension can cause reversible sensorineural hearing loss in children. Early diagnosis and treatment of this rare condition are crucial for hearing recovery.
Area of Science:
- Neurology
- Otolaryngology
Background:
- Idiopathic intracranial hypertension (IIH) is a rare condition characterized by increased intracranial pressure without a clear cause.
- While commonly associated with headaches and visual disturbances, its presentation in pediatric patients can be varied.
Observation:
- A 9-year-old boy presented with progressive bilateral sensorineural hearing loss, headaches, otalgia, tinnitus, and vertigo.
- Audiometry revealed declining bone conduction thresholds over two months.
- Fundoscopy and imaging were normal, but lumbar puncture confirmed elevated intracranial pressure.
Findings:
- The patient was diagnosed with idiopathic intracranial hypertension.
- Following lumbar puncture, his otological symptoms resolved, and hearing returned to normal levels.
- This case highlights a rare pediatric presentation of IIH with reversible hearing loss.
Implications:
- Sensorineural hearing loss in children, accompanied by otological symptoms, should prompt consideration of idiopathic intracranial hypertension.
- Prompt diagnosis and management of IIH can lead to the reversal of hearing deficits.
- This case underscores the importance of a comprehensive workup for pediatric hearing loss, considering neurological causes.
Objective:
To present the rare case of a young boy with idiopathic intracranial hypertension presenting with bilateral sensorineural hearing loss developing over several months. This was accompanied by headaches, otalgia, tinnitus, and vertigo. Furthermore, we aim to provide a concise review on this matter, as this report represents the second case in literature of pediatric idiopathic intracranial hypertension presenting with hearing loss.
Methods:
Workup of a 9-year-old boy with bilateral sensorineural hearing loss, including (among others) physical examination, audiometry, diagnostic imaging, and lumbar puncture.
Results:
Physical examination including fundoscopy as well as imaging showed no abnormalities. At presentation, pure tone audiometry revealed bone conduction thresholds of about 30 dB HL in both ears. Two months later, this declined to about 35 dB HL in both ears. Lumbar puncture revealed an increased intracranial pressure. The boy was thus diagnosed with idiopathic intracranial hypertension. After the lumbar puncture, the otological complaints gradually resolved, and the hearing normalized (bone conduction thresholds of 0-5 dB HL).
Conclusion:
Although rare, sensorineural hearing loss in the pediatric population together with otalgia, tinnitus, and vertigo can be due to idiopathic intracranial hypertension and as such can be reversible.
