There are many causes of hearing loss, some environmental, and some due to genetics. A 30-year-old woman now has persistent ringing in one ear, sometimes enough to disturb hearing conversations in noisy settings. She sees her mother aged 60 is having more and more difficulty hearing almost all the time. She wonders if there is help for both of them.
Progressive hearing loss often begins with such ringing, known as tinnitus. One form of genetics link, passed on to children as a recessive trait, leaves nerves in the ear more susceptible to problems as years go on. In most cases, a gradual reduction in hearing capacity is invisible to others. Nothing outwardly indicates an inability for a person to keep up with conversations. People speak louder not understanding the problem is often one of context, not just volume.
The good news is in genetically-related cases – also termed nonsyndromic – hearing loss is usually treatable, for the mother and the daughter and their loved ones.
Helping Families Understand
Soriya Estes, AuD, co-owner of Estes Audiology with offices in Austin, Boerne, Marble Falls, New Braunfels, and Seguin, TX, specializes in adult aural rehabilitation. Addressing hearing needs, including helping patients and their families, is a unique science. “Hearing aids are not a cure that make things 100 percent right away,” Estes says. “I don’t sell hearing aids; I treat hearing loss.”
Why the distinction? Many people think of glasses and hearing aids as similar appliances. With vision, eyes are tested, prescriptions created, glasses fitted, and people see better right away. With hearing, testing and prescriptions are also part of the diagnosis. Complete treatment, especially in genetically-related cases, involves interviewing the patient and family members. Also, there are a few weeks of adjustment after the start of hearing aid use as the patient becomes reacquainted with hearing.
One question Estes asks: are family members aware of how to talk to someone with hearing loss? Speaking loudly from another room, talking to a person not making eye contact, mumbling with a hand or a beard over a mouth, asking an out-of-the-blue question, and other behaviors we often take for granted can lead to frustration for family members. They also increase embarrassment and isolation for those not hearing fully.
Families linked by genetics are in this together. Estes relates a story about treating generations of one family. Six females all developed hearing issues starting in their 30s, and one of their sons was diagnosed with issues earlier at age 9. Once some family members begin experiencing hearing issues, the likelihood blood relatives will encounter similar challenges is significant.
Genetics and ‘The Slope’ of Change
Individuals are very different in how they hear and perceive speech; a professional audiologist should explore each situation to determine severity, cause, and proper treatment. “Normal” hearing is a somewhat misleading concept as listeners often adapt to mild hearing loss, but moderate to severe cases require attention.
In our case, the 60-year-old mom quips that her hearing problems are likely due to numerous ear infections and too many Van Halen rehearsal sessions in her youth. While any stress on a genetically-weakened ear is not good, damage from environmental factors is telltale and very different from genetic tendencies.
Estes has a sound-proof booth with a computer-driven audio testing system in her New Braunfels office. Testing is a three-step process. First comes a series of tones, without any background noise. This results in the familiar frequency plot spanning from 20 Hz to 20 kHz. Listeners beyond adolescence typically lose sensitivity at the upper end of the range, but otherwise should present a near-flat profile with normal volume response.
Noise-induced impairment such as from loud music or machinery results in a plot with a deep V-shaped notch, a region where almost nothing can be heard, around 3 to 4 kHz. Those frequencies are where a human ear resonates based on cavity size, with moderate to severe mechanical damage often sustained. The impairment may be in one or both ears depending on circumstances.
Genetic hearing loss is often nerve related. Instead of a near-flat profile, a pronounced sloping decline is observed starting at relatively low frequencies and worsening in the upper ranges. Again, left and right ears may be different. Listeners tend to hear lower pitched voices better, but still have difficulty picking out some words. Note both these curves are near or below the “normal” area shaded in light gray.
Deeper Testing Simulates the Real World
The second test targets that difficulty: a word recognition test without background noise. Vowels are typically in base tones, while consonants are in higher ranges. Sibilant sounds such as ‘sh’ are complex and hard to distinguish. With only audio input and no ability to “cheat” using lip reading and preceding words, recognition of random words suffers and guessing begins.
In the third test, background noise is added to word recognition. Noise is increased to the point where it is equally as loud as the spoken words. This simulates a restaurant, sporting event, or any gathering with many people all speaking at once. Mom’s recognition in the right ear fell to 68% at a much lower noise level than expected, compared to a left ear at 92%. Both ears showed more problems with more noise. This highlights hearing is a function of both volume and clarity, involving a lot of processing.
Part II of this blog looks at mom’s treatment, and testing and treatment for the daughter.