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Old 10-26-2006, 07:16 AM
leaf leaf is offline
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Join Date: Oct 2006
Location: Bristol UK
Posts: 45

I was surprised at the number of people with both meniere's and HNPP, not everyone but even finding one other with both in a group of 130 people would have been a surprise. But there were at least 6 others, and then there were those with hearing loss and tinnitus or and other forms of hearing loss such as Auditory neuropathy.
I have tried to discuss this with my Doctors but they are so blinkered. I may as well talk to a clod of earth, at least it wouldn't be patronising and arrogant.

There have been several reports in med literature pointing out this combination, most recently this one,


Otol Neurotol. 2005 May;26(3):405-14.

Sensorineural hearing impairment in patients with Pmp22 duplication, deletion, and frameshift mutations.

* Verhagen WI,
* Huygen PL,
* Gabreels-Festen AA,
* Engelhart M,
* van Mierlo PJ,
* van Engelen BG.

Department of Neurology, Canisius-Wilhelmina Hospital, Nijmegen, The Netherlands.

OBJECTIVE: To characterize and distinguish the types of sensorineural hearing impairment (SNHI) that occur in hereditary motor and sensory neuropathy Type 1a (HMSN-1a) and hereditary neuropathy with liability to pressure palsies (HNPP), which are caused by deletion or frameshift mutation. STUDY DESIGN: Prospective study. SETTING: Ambulatory patients in a university hospital. PATIENTS: Twelve patients with HMSN-1a due to a duplication of the PMP22 gene on chromosome 17p11.2, 16 patients with HNPP due to the common PMP22 deletion (HNPP del), and 11 HNPP patients with a frame shift mutation (heterozygous PMP22 G-insertion) (HNPP mut), all confirmed by molecular genetic analysis. INTERVENTIONS: Pure-tone audiograms and speech audiograms were obtained. MAIN OUTCOME MEASURES: Results of cross-sectional analysis comprising linear regression of hearing threshold on age. RESULTS: Pure-tone audiograms showed mild to moderate SNHI, predominant at the low and the high frequencies. SNHI showed significant progression by approximately 0.4 dB per year at 0.25 to 4 kHz and up to 1 to 2 dB per year at 4 to 8 kHz. Patients with HMSN-1a had substantial, presumably congenital, SNHI but did not show significant progression beyond presbyacusis. Patients with HNPP showed postnatal onset at age 11 years with progression of SNHI in excess of presbyacusis by 0.4 dB per year. All three types of neuropathy showed normal speech recognition. CONCLUSIONS: All three types of neuropathy showed SNHI with normal speech recognition. HMSN-1a showed stable SNHI without progression beyond presbyacusis. HNPP showed progression beyond presbyacusis with postnatal onset. The differences in SNHI may be explained by the differences in PMP22 expression. The progressive SNHI in HNPP might be explained by the liability for exogenous factors associated with this disorder.

PMID: 15891642 [PubMed - indexed for MEDLINE]
I think the conclusions hit the nail firmly on the head.

I've tried numerous medical treatments, and even surgery (saccus decompression) to control the vertigo, all with limited success. I use cinnarizine as a vestibular sedative and to reduce nausea, it is also supposed to have some action on the fluid balance of the inner ear by it's calcium channel blocking action. I think in the US it's stronger and longer-lasting derivative is sometimes used - Flunarizine. It's a type of anti-histamine, but with other effects. It can also be used as a Migraine preventative, though not very often.

Do you also suffer from Migraine? I wondered whether this might be the provoking factor for some.

HNPP - Hereditary Neuropathy with liabilty to pressure palsy.
HNPP UK Links and Info
Hnpp Info page : CMTA : CMT UK
Inner Ear Disease
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