can ear surgery reclaim some of the hearing loss resulting from osteogenesis imperfecta?

Question

i hav osteogenesis imperfecta. as a result, i started having hearing loss at around 25 years. now, some drs. recommend ear surgery which can reclaim some of the loss, they say. they are all ENTs. they speak from their point of view.

can someone with experience in orthopaedics advice from their point of view if surgery wud prove beneficial?

also, is it possible that the surgery may break the soft bones of the ear since they are so soft, or the bones cant hold the artificial bone that wud be inserted?

please advice

Answer 1

Hi, I'm an Audiologist. I know you want to hear from Orthopedic people, but I'll add my input, too. For one thing, the orthopedic specialists don't do stapedectomies, because it is such a delicate procedure, so their experience will be mostly limited to what they've heard - and in the medical field we hear complaints far more than praises, because the patients that are happy have no reason to come in and see us.

The stapedectomy procedure does have risks involved, and I'd guess that the osteogenesis imperfecta complicates things a bit more. As you point put, I would think that part of the issue will be long-term retention of the prosthesis.

Here's the Osteogenesis Imperfecta Foundation page on hearing loss: http://www.oif.org/site/PageServer?pagename=HearLoss
This page discusses how the different types of OI have different implications: http://www.oif.org/site/PageServer?pagename=TypeI

"Individuals with conductive loss tha tis severe and progressive may be helped with a surgical procedure known as a stapedectomy. In this procedure, the fixed foot process of the stapes is replaced by a prosthesis that allows for the normal propagation of sound waves to the inner ear. It should be noted, however, that this surgery should not be considered routine in OI because of tissue fragility. There are also many other pre- and post-operative issues that need to be assessed, discussed, and clarified before any individual with OI may be considered a "good candidate" for surgery. As a general rule, patients should seek treatment centers where the otologists (doctors with a subspecialty in ear disorders) have considerable experience with stapes surgery."
From: http://www.osteo.org/newfile.asp?doc=r604i&doctitle=Hearing+Loss+%26+Bone+Disorders&doctype=HTML+Fact+Sheet

"A majority of adults with osteogenesis imperfecta have functionally significant hearing loss related to combined conductive and sensorineural deficits. [11] In most cases, deficits are detectable only on audiology examination in childhood and the teen years; functional loss does not occur until the twenties. A study of hearing loss in Finnish children with OI reported an incidence of 6.7% with loss greater than 20 dB [12]; this is comparable to the 7.7% detected in the NIH pediatric OI population. [13] Most pediatric hearing loss is detected between ages 5-9 years; some children may require hearing aids. For adults, the hearing deficits are very similar to those found in otosclerosis. Surgical intervention with stapedectomy can give satisfactory long-term results when hearing loss exceeds the compensation of hearing aids. However, this surgery should not be undertaken routinely. The fragility of the small bones of the ear results in a significant percentage of unsatisfactory long term hearing restoration, even in experienced hands. [14]"
From: http://www.endotext.org/parathyroid/parathyroid17/

Other surgical options depend on the type of hearing loss, as well as your individual case. A Cochlear Implant is for sensorineural hearing losses, and since the stapedectomy is currently being recommended to you, your hearing loss must be primarily conductive; therefore the Bone Anchored Hearing Aid (BAHA) would be more audiologically appropriate. Again - because it is an implant in the skull, the individual patient's bone density is the primary concern in determining eligibility.

This study in 2001 had success implanting the BAHA in 40 patients (only one had complications) -- of course, they were carefully selected for eligibility.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=11347635&dopt=Abstract

I would strongly suggest consulting a MAJOR otology center for such a procedure - for example, John's Hopkins, the Mayo Clinic, Vanderbilt University, House Ear Institute. That's where you will get the best and most experienced surgeons, especially with challenging cases like yours.

Otherwise, hearing aids are an option, depending on the degree of your loss. A conductive hearing loss is typically the best candidate for amplification, because the cochlea and auditory nerve are still functioning normally.

Feel free to click on my name/avatar and then "contact HearKat" to send me a note if you want to. Good luck to you!

Answer 2

I have had ear surgery and I have some concerns now. I had the surgery in December 2013 and since that Ive had bad taste buds. Everything taste bad.My doctor keeps saying that it should go away but its been 8 months and I still have the situation.

Answer 3

WHAT? WHAT DID YOU SAY?