Born with hearing loss

Hearing loss is usually talked about as something that happens later in life. For many people, it's there from the start. In the U.S., newborn screening identified permanent hearing loss in about 1.7 of every 1,000 babies screened in 2022, more than 6,000 infants that year.¹ More children are identified later, because some hearing loss appears after birth or is too mild to catch at first.

Newborn hearing screening

More than 98% of babies born in the U.S. have their hearing screened,¹ usually before they leave the hospital. It takes a few minutes, doesn't hurt, and is often done while the baby sleeps. There are two methods, both described in the rest of a hearing test:

• Otoacoustic emissions (OAE): a soft probe in the ear canal listens for the faint sounds a healthy cochlea makes in response to sound.
• Automated auditory brainstem response (AABR): small sensors on the head measure the nerve's response to soft clicks.

A refer result doesn't mean a baby has hearing loss. Fluid or debris in the ear canal after birth is a common reason. It does mean the baby needs a follow-up test. U.S. programs aim for 1-3-6: screening by 1 month of age, a full diagnostic evaluation by 3 months, and early intervention services by 6 months.²

Passing the screening isn't a guarantee for life. Screening can miss mild loss and loss in one ear, and some hearing loss develops or progresses during childhood.³ If a parent, teacher, or the child notices something, it's worth a full hearing test at any age.

Common causes

At least half of hearing loss present at birth is genetic. The most common genetic cause is a change in a gene called GJB2, which makes a protein called connexin 26. It often appears in families where both parents hear typically, because each can carry one copy of the variant without any hearing loss of their own.⁴ Most genetic hearing loss involves hearing only, but some is part of a syndrome that can affect vision, balance, or other parts of the body, which is one reason genetic testing can be useful.⁵

The most common cause that isn't genetic is congenital cytomegalovirus (cCMV), a common virus passed to a baby before birth. Hearing loss from cCMV can be present at birth or appear and progress over the first years of life, and some states now test newborns for it.⁶ Other causes include premature birth, some infections, and some medications given to newborns.

Language from the start

About 90% or more of deaf and hard of hearing children are born to hearing parents.⁷ What matters most in the early years is full access to language: a spoken language, a signed language such as American Sign Language (ASL), or both. Many families use both, and choosing one path early doesn't close the other. In the U.S., early intervention services are available from birth to age 3, with support at school after that.⁸

Hearing technology

Hearing aids can be fitted in the first months of life, and children's hearing aids are adjusted as the ear canal grows. For babies with profound hearing loss in both ears, cochlear implants are approved in the U.S. from 9 months of age.⁹ Families make these choices, and later the person does. Many Deaf adults don't use hearing technology at all, and live full lives in sign language.

Growing up with hearing loss

Many people who grew up hard of hearing describe some of the same things:

• Not knowing what you don't hear. If birdsong or the s in a word was never there, there's nothing to notice is gone. An audiogram, or hearing typical hearing in the simulator, can be a surprise even to people who have had hearing loss all their lives.
• Listening fatigue. Following speech takes constant effort, filling in gaps from context and lips, so a long day of conversation can be exhausting in a way hearing people often don't see.
• Lipreading without being taught. Many people learn to read lips on their own, and only realize how much they rely on it when someone turns away, covers their mouth, or wears a mask.
• Hearing that seems to come and go. How much you understand changes with noise, distance, lighting, and how tired you are. It isn't selective hearing, even when it's mistaken for it.
• Being in between. Hard of hearing people can feel they don't fully belong in hearing spaces or in Deaf spaces. Many find community in both, and with each other.

Words people use

Deaf, with a capital D, refers to a cultural and linguistic community, often built around a signed language. deaf, lowercase, describes hearing level. Hard of hearing is used by many people with mild to severe hearing loss who mainly use spoken language. Most people in these communities prefer these terms to hearing impaired, which many find focuses on what's wrong rather than who they are. The best term for any one person is the one they use for themselves.¹⁰