From the Guidelines
Idiopathic CSF hypertension can affect hearing through several mechanisms related to elevated cerebrospinal fluid pressure, and patients may experience hearing disturbances including tinnitus, aural fullness, low-frequency hearing loss, and occasionally sudden sensorineural hearing loss, as noted in studies such as 1.
Key Points
- The connection between idiopathic CSF hypertension and hearing dysfunction relates to the continuous fluid pathway between CSF and perilymph, allowing pressure changes to affect cochlear function.
- Management of hearing symptoms in idiopathic CSF hypertension primarily focuses on treating the underlying condition through weight loss (if applicable), acetazolamide, or topiramate.
- For severe cases unresponsive to medical therapy, CSF diversion procedures like lumboperitoneal or ventriculoperitoneal shunting, or optic nerve sheath fenestration may be necessary.
- Regular audiometric monitoring is recommended for patients with hearing complaints, as hearing symptoms may become permanent if elevated pressure persists for extended periods, and rehabilitation options such as hearing aids or assistive listening devices may be beneficial for patients with residual hearing loss or tinnitus, as discussed in 1 and 1.
Treatment and Monitoring
- Acetazolamide can be started at 500mg twice daily, increasing as needed up to 2-4g daily.
- Topiramate can be started at 25-50mg daily, titrating up to 100-200mg daily.
- Repeated audiometric assessment with continued follow-up is needed to monitor hearing stability and assess the benefit of interventions.
- The significant impact of unilateral sensorineural hearing loss on patients’ quality of life has been shown in adults and children, and rehabilitation options should be discussed with patients to alleviate anxiety and improve outcomes, as noted in 1.
From the Research
Idiopathic CSF Hypertension and Hearing
- Idiopathic intracranial hypertension (IIH) is characterized by increased intracranial pressure in the absence of neurologic tumors 2.
- Patients with IIH may experience tinnitus, hearing loss, balance disturbance, cerebrospinal fluid (CSF) otorrhoea or rhinorrhoea, and in some cases, these otorhinological symptoms can be presenting features of this syndrome 3.
- The exact relationship between IIH and hearing loss is not fully understood, but it is thought to be related to the increased intracranial pressure and its effects on the auditory system 3.
- Acetazolamide, a carbonic anhydrase inhibitor, is effective in reducing intracranial pressure and may be used to treat IIH, including cases with associated hearing symptoms 2, 4.
- Studies have shown that IIH patients may have abnormal CSF dynamics, including increased CSF pressure, resistance to CSF outflow, and sagittal sinus pressure, which may contribute to hearing symptoms 5.
Treatment and Management
- Treatment of IIH typically involves reducing intracranial pressure, reducing pain, and protecting the optic nerves 6.
- Acetazolamide is a common treatment for IIH, and its effectiveness in reducing intracranial pressure has been demonstrated in several studies 2, 4, 5.
- Surgical intervention may be necessary for patients experiencing visual loss or impending visual loss and not responding to medication therapy 6.
- Further research is needed to understand the relationship between IIH and hearing loss, as well as to develop effective treatment strategies for patients with IIH and associated hearing symptoms 3, 5.