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Adults with Mito-related Hearing Loss

Question: 

I just had a ”routine”  hearing test and was told that I have a moderate to severe sensorineural hearing loss in both ears.  I had noticed that the radio, TV, and telephone were sounding a bit muffled, but I was shocked to find out that my hearing was that bad!  How could I lose so much without realizing it?  I have a niece who has Mito and was born deaf.  She had cochlear implants placed when she was a toddler, and she goes to a school for the deaf that uses both sign language and spoken English for communication.  Should I learn sign language, even though she’s the only person I know who signs?   My Mito doctor said that hearing loss is very common, especially for people with MELAS, and that it is most likely progressive.  I have an appointment to be fitted with hearing aids, but my health insurance doesn’t cover them and I don’t know how I will pay for them.  How will I use the phone if I can’t hear what the caller is saying?   I have hundreds of questions and don’t know how to find the answers.  

~A.L. in Massachusetts

Answer: 

It sounds like you entered the audiologist’s office expecting to complete a routine test, but ended up receiving a frightening and isolating diagnosis.  Hearing loss is a very common medical finding; in fact, 1 in 5 adults in the US has a clinically significant hearing impairment.  Hearing loss is also a common feature found within individuals with Mitochondrial disease.  According to some studies, the rate of mild to profound bilateral sensorineural hearing loss in adults with genetically confirmed Primary Mito could be as high as 60%.  The prevalence of hearing loss in MELAS and MIDD is 78% to 95%.  Adult-onset hearing loss is an “invisible disability;” the effects may not be visible to the casual observer, but they may be devastating to the patient’s quality of life.  When hearing loss or deafness is discovered in infancy or childhood, the child generally receives special education services, and learns to communicate via American Sign Language, oral English with hearing aids or Cochlear Implants, or a combination of methods. People who lose their hearing as a teen or adult, having already established oral/aural English as their method of communication, are considered “Late Deafened”.  Many late deafened adults feel like they are trapped between two worlds: they do not identify with the unique language and culture of the Deaf Community, yet their hearing loss interferes with their communication abilities to such an extent that they have great difficulty functioning in the hearing world.  Unfortunately, many late-deafened adults do not receive any guidance related to communication strategies, availability of ADA accommodations, financial assistance, or social/emotional support following their hearing loss diagnosis.   The Massachusetts Commission for the Deaf and Hard of Hearing has provided a very informative e-book for Late Deafened Adults: The Savvy Consumer’s Guide to Hearing Loss”  by Karen Rockow, Ph.D.

Here are some resources for newly-diagnosed, late-deafened adults:

Communication:  For many adults, one of the first indications of hearing loss is difficulty understanding phone conversations.  In August of 2011, Sorenson Communications announced the release of CaptionCall, a state-of-the-art device that is fully compatible with standard landline telephone services, but also provides nearly-instantaneous captions similar to the process used during live TV broadcasting.  Sorenson Communications provided the following description of the system in a press release:

Similar to captioned television, CaptionCall uses innovative voice recognition technology and a transcription service to quickly display written captions of what callers say on a large, easy-to-read screen. The free, Federal Communications Commission (FCC)-funded service is delivered through the state-of-the-art CaptionCall telephone, which works like a regular phone. Customers make and receive calls as they always have.  All that is required to use CaptionCall is a high-speed internet connection, a standard home phone line and an ordinary home electrical outlet. 

 "Virtually anyone with hearing loss faces the challenge of communicating by phone," notes Sorenson Communications President and CEO Pat Nola. "The ability to communicate by phone empowers people to stay connected, remain independent and call with confidence. CaptionCall helps keep personal and business relationships alive and vibrant."www.CaptionCall.com
 

[Note: there are several other communication methods available to individuals with hearing, speech, vision, and motor disabilities.  Look for the article :Today’s Options for Adaptive Communication; which is expected to be published on MitoAction in December 2012.]

Environmental Sounds:  Most individuals who wear hearing aids remove them at bedtime.  Unfortunately, they may be unable to hear a standard alarm clock, or even a smoke or carbon monoxide detector. Several manufacturers offer alarm clocks that vibrate, flash lights, or both.  Some alarm clocks can be adjusted to produce a tone that is audible to the sleeper even without hearing aids. Alarm clocks  Smoke, fire, and carbon monoxide detectors with strobe lights and alarms above 110dB.  They are available from several companies.  You can find examples of these devices here.  If you live in an apartment, your landlord is required to install smoke and carbon monoxide alarms with a strobe light.  This link  provides more details about ADA compliance for landlords and employers.

Transportation:  Hearing loss does not affect driving ability.  In fact, many sources report that deaf or hard of hearing drivers tend to be more visually alert and less distracted by sounds, putting them at an advantage over drivers who can hear.  However, Deaf and hard of hearing drivers may be unable to hear a law enforcement officer’s instructions during a routine traffic stop.  The officer has no way of knowing your hearing status as he/she approaches your vehicle; reaching for something on the floor or rummaging through a purse could be misinterpreted as aggressive behavior, with tragic results.   The Center for Hearing Loss Help has created informational cards that are placed on your vehicle’s sun visors.  The visor cards and detailed information regarding their use can be found in this article.   

Weather Safety  Many people are accustomed to listening to the weather on a car radio, or by watching the live broadcast morning news or a video on a news website. Unfortunately, most local news channels do not provide captions during the weather report and some sports segments.   Luckily, there are services and devices available to keep you informed of weather or other catastrophic emergencies.

The Massachusetts Emergency Management Agency (MEMA) encourages smartphone users to download the free ping4alerts! app. This app is available free of charge for iPhone and Android phones and receives severe weather alerts from the National Weather Service and emergency information from MEMA based on the user's location.  Learn more at  http://www.mass.gov/mema/mobileapp. 

If you do not live in Massachusetts, the Federal Emergency Management Agency (FEMA) offers text messages with emergency information, shelter location, and National Weather Service updates.  The service can be found here.

The NOAA Weather Radio monitors for weather emergencies and other broadcasts (nuclear emergencies, AMBER alert, etc.)  This is a brochure for the standard model.  You can also purchase a NOAA radio that alerts with flashing lights and the message is converted into text and scrolled across a screen.  The NOAA Weather Radio for the deaf and hard of hearing can be found here.

 The American Red Cross offers mobile apps that provide  information and guidance regarding first aid, wildfires, earthquakes, hurricanes, and shelter locations. For more information, contact the American Red Cross website.

Assistance and Case Management: Every state in the US has a commission for the deaf and hard of hearing.  Contact information for your particular state can be found here.   In most circumstances, eligibility for case management services is determined by the impact hearing loss has on the individual’s daily living, rather than the results of an audiogram.    

a.    Equipment such as smoke alarms and doorbells: lending library and financial assistance

b.    Vocational Rehabilitation Programs, providing accommodation for current employment or additional training/education if a career change is necessary.                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                 

c.    Assistance securing health care, including testing, hearing aids or cochlear implants

d.    Sign language classes (ASL, Signed Exact English, Sign-Supported English)

e.    Speechreading (oral deaf communication); group and individual instruction

f.     Support and assistance with Social Security Disability or SSI applications

 Hearing loss is a bit like “the elephant in the room;” frequently encountered and rarely discussed.  According to Empowerment Through Hearing,Over 35 million Americans have a significant hearing loss. Yet, 80% of those diagnosed do not seek help. This is due in part to denial, embarrassment or lack of awareness. We don’t know what we don’t hear.” 

 

Hearing loss is frequently misdiagnosed as depression, anxiety, and even cognitive decline.  Among people with Mitochondrial Disease, more than half of us are deaf or hard of hearing.   Follow http://www.MitoAction.org  for more “Cut The Red Tape!” articles, offering support to people with vision and hearing loss related to Mitochondrial Disease.

 

Please read more about Mito and Hearing loss here.

 

Comments

cswan's picture

cswan

04/01/2013

I totally relate, I too lost my hearing later, started about 41 yrs old. And I am still lost with things. Last year I got a cochlear implant. I had NO hearing at all.The cochlear is great for one on one,and is better then what I had, but voices are still muffled, I still use cc for tv. radio is just a horrible noise.
They will only cover us here in Canada for 1 implant which I am very thankful for. I wish we could have 2 as I do feel off balance. But 1 is better then nothing.
I have bed shakers,door flasher and all the stuff to alert me. I have now been told that an FM receiver may be good for me. I am now researching that as I have no idea how it works or anything.
If u know anything about the FM can u please message me?
thanks