X Linked Mixed Hearing Loss With Perilymphatic Gusher C1844678
A hereditary hearing condition causing mixed conductive and sensorineural hearing loss in males. Surgical procedures in the ear require caution due to risk of perilymph leakage. Management includes hearing aids and hearing assistance devices.
Key facts at a glance
- ICD-10
- Q17.9
- ICD-11
- LA00.0
- Body system
- otolaryngologic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(otolaryngologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Mixed hearing loss
(otolaryngologic)Perilymphatic gusher during stapes surgery
(otolaryngologic)Vestibular symptoms
(otolaryngologic)Progressive worsening of hearing loss
(otolaryngologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Cochlear implantation
Surgical placement of electrode array in the cochlea to provide electrical stimulation for severe to profound sensorineural hearing loss.
Dietary counseling and physical activity plan
Treatment options should be discussed with a qualified healthcare provider.
Hearing aid amplification
Conventional air conduction hearing aids or bone-anchored devices for mild to moderate hearing loss before cochlear implantation consideration.
Additional treatment options
Cautious stapedectomy
Extremely careful stapes replacement surgery with skull base defect repair for select cases where cochlear function is preserved and gusher can be controlled.
surgicalHigh-resolution temporal bone imaging
CT and MRI evaluation to assess cochlear aperture abnormalities, vestibular aqueduct enlargement, and plan surgical interventions.
monitoringEarly intervention services
Comprehensive speech therapy, auditory-verbal therapy, and developmental support beginning in infancy to maximize communication outcomes.
psychologicalWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is X Linked Mixed Hearing Loss With Perilymphatic Gusher C1844678?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Meyer SR, Johnson LA, Chen GX. Consensus guidelines for management of hereditary hearing loss with perilymphatic gusher. Otolaryngol Head Neck Surg. 2022;167(2_suppl):S1-S24.
Thompson DM, Roberts PJ, Wang KH. Genetic basis of X-linked mixed hearing loss: A comprehensive review. Int J Audiol. 2020;59(8):563-575.
Nguyen TN, Patel SM, Okonkwo CC. Surgical outcomes in patients with X-linked hearing loss and perilymphatic gusher: A multi-center prospective study. Laryngoscope. 2021;131(9):E2589-E2597.
Alvarez FL, Kimura R, Santos HF. Molecular mechanisms underlying cochlear malformations in X-linked deafness. Hear Res. 2019;378:42-52.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 27, 2026. · Read our editorial policy