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Neurofibromatosis Type II

WHAT IS NEUROFIBROMATOSIS TYPE II?

Neurofibromatosis Type II, (NF2) also known as Multiple Inherited Schwannomas, Meningiomas and Ependymomas (MISME) is a genetically inherited disease. It is distinguishable from NF Type 1, also known as von Recklinghausen’s disease. The primary manifestation of NF2 is a development of non-malignant (benign) brain tumors in the region of the cranial nerves, frequently bilaterally. The eighth cranial nerve is the auditory-vestibular nerve which transmits sensory information from the inner ear to the brain and is commonly affected.

NF II is caused by mutations of the "Merlin" gene, which appears to influences the form and movement of cells.

WHAT ARE THE SYMPTOMS OF NEUROFIBROMATOSIS TYPE II?

Individuals who suffer with NF2 may develop a wide range of distinct problems including:

  • Hearing loss;
  • 90% of patients show bilateral acoustic neuromas through tympanography or magnetic resonance imaging. (MRI) testing;
  • 50% of patients develop tumors on the cranial nerves called meningiomas;
  • 50% of patients develop spinal lesions;
  • 90% of patients suffer eye lesions. In teenagers, there can be recurrent cataracts
  • Facial palsy (paralysis of the seventh cranial nerve which controls eyelid function).

WHAT IS THE TREATMENT FOR NEUROFIBROMATOSIS TYPE II?

The first step of treatment is to undergo a comprehensive medical examination by an Otologist and Neurosurgeon in order to determine if there is a Neurofibromatosis type II is present. NF2 can be conclusively diagnosed by blood test. Surgery, radiation therapy, observation, and partial debulking of tumors have all been used with effect. In certain cases, hearing loss can be treated with Auditory Brainstem implants similar to Cochlear Implants. Specific cranial nerve deficits require specialized treatment such as eyelid gold weight implant for eye problems, reconstruction of vocal cord positioning for hoarseness or swallowing difficulties, hearing aids, and other methods.

WHAT IS THE MANAGEMENT FOR HEARING LOSS IN NF2?

Individuals who have been diagnosed with NF2 have almost always already acquired speech and language skills before the tumors present a problem. Therefore, they do not always integrate well into the deaf culture and are most likely to choose assistive auditory technology. Cochlear implants and auditory brainstem implants are the two technologies which can restore functional hearing to the severely-to-profoundly deaf individuals with NF2.

CONCLUSION:

Diagnosis for NF2 requires a comprehensive medical examination and specialty care from an Otologist, who is an otolaryngologist (ENT) who has undergone two additional years of training and specializes in issues related to the nerves of hearing only. With appropriate medical treatment and therapy, individuals with NF2 can make a partial or even full recovery.

Click here to make an appointment with the California Ear Institute to consult with one of our board certified Otologists regarding your condition.

 

 

 

 


 
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