Hearing Loss

1. 1. Pham NS: The management of pediatric hearing loss caused by auditory neuropathy spectrum disorder. Curr Opin Otolaryngol Head Neck Surg 25(5):396-399, 2017. doi: 10.1097/MOO.0000000000000390

1. 1. Liberman MC, Kujawa SG: Cochlear synaptopathy in acquired sensorineural hearing loss: manifestations and mechanisms. Hear Res 349:138-147, 2017. doi: 10.1016/j.heares.2017.01.003

Most adults and older children notice a sudden hearing loss, and caregivers may suspect that a neonate has a severe hearing loss within the first weeks of life when the neonate does not respond to voices or other sounds. However, progressive losses and nearly all losses in infants and young children must be detected by screening. Screening in children should begin at birth so that linguistic input can allow optimal language development. If screening is not done, severe bilateral losses may not be recognized until age 2 years, and mild to moderate bilateral or severe unilateral losses are often not recognized until children reach school age.

Screening in older people should be considered because patients may not have noticed a gradual decline in hearing or may think it a normal consequence of aging.

Suspected hearing loss at any time should prompt referral to a specialist.

History of present illness should note how long hearing loss has been perceived, how it began (eg, gradual, acute), whether it is unilateral or bilateral, and whether sound is distorted (eg, music is off—dull or lifeless) or there is difficulty with speech discrimination. The patient should be asked whether the loss followed any acute event (eg, head injury, loud noise exposure, barotrauma [particularly a diving injury], or starting of a drug). Important accompanying symptoms include other otologic symptoms (eg, ear pain, tinnitus, ear discharge), vestibular symptoms (eg, disorientation in the dark, vertigo), and other neurologic symptoms (eg, headache, weakness or asymmetry of the face, an abnormal sense of taste, fullness of the ear). In children, important associated symptoms include presence of delays in speech or language development, visual changes, or delayed motor development.

Review of systems should seek to determine the impact of hearing difficulty on the patient’s life.

Past medical history should note previous possibly causative disorders, including central nervous system infection, repeated ear infections, chronic exposure to loud noise, head trauma, rheumatic disorders (eg, rheumatoid arthritis, lupus), and a family history of hearing loss. Drug history should specifically query current or previous use of ototoxic drugs. For young children, a birth history should be sought to determine if there were any intrauterine infections or birth complications.

The focus is examination of the ears and hearing and the neurologic examination. The external ear is inspected for obstruction, infection, congenital malformations, and other lesions. The tympanic membrane (TM) is examined for perforation, drainage, otitis media (pus or fluid seen in the middle ear through the TM), and cholesteatoma. During the neurologic examination, particular attention needs to be paid to the 2nd through 7th cranial nerves as well as to vestibular and cerebellar function because abnormalities in these areas often occur with tumors of the brain stem and cerebellopontine angle. The Weber and Rinne tests require a tuning fork to differentiate conductive from sensorineural hearing loss.

In the Weber test, the stem of a vibrating 512-Hz or 1024-Hz tuning fork is placed on the midline of the head, and the patient indicates in which ear the tone is louder. In unilateral conductive hearing loss, the tone is louder in the ear with hearing loss. In unilateral sensorineural hearing loss, the tone is louder in the normal ear because the tuning fork stimulates both inner ears equally and the patient perceives the stimulus with the unaffected ear.

In the Rinne test, hearing by bone and by air conduction is compared. Bone conduction bypasses the external and middle ear and tests the integrity of the inner ear, 8th cranial nerve, and central auditory pathways. The stem of a vibrating tuning fork is held against the mastoid (for bone conduction); as soon as the sound is no longer perceived, the fork is removed from the mastoid, and the still-vibrating tines are held close to the pinna (for air conduction). Normally, the fork can once more be heard, indicating that air conduction is better than bone conduction. With conductive hearing loss over 25dB, the relationship is reversed; bone conduction is louder than air conduction. With sensorineural hearing loss, both air and bone conduction are reduced, but air conduction remains louder.

Findings of particular concern are

• Unilateral sensorineural hearing loss

• Abnormalities of cranial nerves (other than hearing loss)

• Rapidly worsening or sudden hearing loss

Many causes of hearing loss (eg, cerumen, injury, significant noise exposure, infectious sequelae, drugs) are readily apparent based on results of the history and examination (see table Some Causes of Acquired Hearing Loss).

Associated findings are helpful in diagnosing the remaining small number of patients in whom no clear cause can be found. Patients who have focal neurologic abnormalities are of particular concern. The 5th or 7th cranial nerve or both are often affected by tumors that involve the 8th nerve, so loss of facial sensation and weak jaw clench (5th) and hemifacial weakness and taste abnormalities (7th) point to a lesion in that area. Signs of autoimmune disorders (eg, joint swelling or pain, eye inflammation) or renal dysfunction may suggest these disorders as a cause. Maxillofacial malformations may suggest a genetic or developmental abnormality.

All children with delays in speech or language development or difficulty in school should be evaluated for hearing loss. Intellectual disability, aphasia, and autism must also be considered. Delayed motor development may signal vestibular deficit, which is often associated with a sensorineural hearing loss.

Testing includes

• Audiologic tests

• Sometimes MRI or CT

Audiologic tests are required for all people who have hearing loss; these tests usually include

• Measurement of pure-tone thresholds with air and bone conduction

• Speech reception threshold

• Speech discrimination

• Tympanometry

• Acoustic reflex testing

Information gained from these tests helps determine whether more definitive differentiation of sensory from neural hearing loss is needed.

Pure-tone audiometry quantifies hearing loss. An audiometer delivers sounds of specific frequencies (pure tones) at different intensities to determine the patient’s hearing threshold (how loud a sound must be to be perceived) for each frequency. Hearing in each ear is tested from 125 or 250 to 8000 Hz by air conduction (using earphones) and up to 4 kHz by bone conduction (using an oscillator in contact with the mastoid process or forehead). Test results are plotted on graphs called audiograms (see figure Audiogram), which show the difference between the patient’s hearing threshold and normal hearing at each frequency. The difference is measured in dB. The normal threshold is considered 0 dB hearing level (Hl); hearing loss is considered present if the patient’s threshold is > 25 dB Hl. When hearing loss is such as to require loud test tones, intense tones presented to one ear may be heard in the other ear. In such cases, a masking sound, usually narrow band noise, is presented to the ear not being tested to isolate it.

Audiogram of right ear in a patient with normal hearing

Normal audiogram of the right ear. The vertical lines represent the frequencies that are tested from 125 to 8000 Hz. The horizontal lines record the threshold at which the patient states that the sound is heard. Normal thresholds are 0 dB +/− 10 dB. Patients with a hearing threshold below 20 dB are considered to have average or better-than-average hearing. The greater the dB, the louder is the sound and the worse the hearing. "O" is the standard symbol for air conduction of the right ear; "X" is the standard symbol for air conduction for the left ear. The "<" is the standard symbol for unmasked bone conduction for the right ear; ">" is the standard symbol for unmasked bone conduction of the left ear. The reason why both masked and unmasked measures are needed is to make sure one ear is not hearing the sound presented to the other ear (one ear is 'masked' so it does not hear the sound presented to the other ear, giving a false value).

Speech audiometry includes the speech reception threshold (SRT) and the word recognition score. The SRT is a measure of the intensity at which speech is recognized. To determine the SRT, the examiner presents the patient with a list of words at specific sound intensities. These words usually have 2 equally accented syllables (spondees), such as "railroad," "staircase," and "baseball." The examiner notes the intensity at which the patient repeats 50% of the words correctly. The SRT approximates the average hearing level at speech frequencies (eg, 500 Hz, 1000 Hz, 2000 Hz).

The word recognition score tests the ability to discriminate among the various speech sounds or phonemes. It is determined by presenting 50 phonetically balanced one-syllable words at an intensity of 35 to 40 dB above the patient’s SRT. The word list contains phonemes in the same relative frequency found in conversational English. The score is the percentage of words correctly repeated by the patient and reflects the ability to understand speech under optimal listening conditions. A normal score ranges from 90 to 100%. The word recognition score is normal with conductive hearing loss, albeit at a higher intensity level, but can be reduced at all intensity levels with sensorineural hearing loss. Discrimination is even poorer in neural than in sensory hearing loss. Testing of words understood within full sentences is another type of recognition test that is often used to assess candidacy for implantable devices (when the benefit from hearing aids is insufficient).

Tympanometry measures the impedance of the middle ear to acoustic energy and does not require patient participation. It is commonly used to screen children for middle ear effusions. A probe containing a sound source, microphone, and air pressure regulator is placed snugly with an airtight seal into the ear canal. The probe microphone records the reflected sound from the tympanic membrane (TM) while pressure in the canal is varied. Normally, maximal compliance of the middle ear occurs when the pressure in the ear canal equals atmospheric pressure. Abnormal compliance patterns suggest specific anatomic disruptions. In eustachian tube obstruction and middle ear effusion, maximal compliance occurs with a negative pressure in the ear canal. When the ossicular chain is disrupted, as in necrosis or dislocation of the long process of the incus, the middle ear is excessively compliant. When the ossicular chain is fixed, as in stapedial ankylosis in otosclerosis, compliance may be normal or reduced.

The acoustic reflex is contraction of the stapedius muscle in response to loud sounds, which changes the compliance of the TM, protecting the middle ear from acoustic trauma. The reflex is tested by presenting a tone and measuring what intensity provokes a change in middle ear impedance as noted by movement of the TM. An absent reflex could indicate middle ear disease or a tumor of the auditory nerve. Any conductive hearing loss abolishes the acoustic reflex. Additionally, facial paralysis abolishes the reflex because the facial nerve innervates the stapedius muscle.

Advanced testing is sometimes needed. Gadolinium-enhanced MRI of the head to detect lesions of the cerebellopontine angle may be needed in patients with an abnormal neurologic examination or those whose audiologic testing shows poor word recognition, asymmetric sensorineural hearing loss, or a combination when the etiology is not clear.

CT is done if bony tumors or bony erosion is suspected. Magnetic resonance angiography and venography is done if vascular abnormalities such as glomus tumors are suspected.

The auditory brain stem response uses surface electrodes to monitor brain wave response to acoustic stimulation in people who cannot otherwise respond.

Electrocochleography measures the activity of the cochlea and the auditory nerve with an electrode placed on or through the eardrum. It can be used to assess and monitor patients with dizziness, can be used in patients who are awake, and is useful in intraoperative monitoring.

Otoacoustic emissions testing measures sounds produced by outer hair cells of the cochlea in response to a sound stimulus usually placed in the ear canal. These emissions are essentially low-intensity echoes that occur with cochlear outer hair cell activation. Emissions are used to screen neonates and infants for hearing loss and to monitor the hearing of patients who are using ototoxic drugs

Central auditory evaluation measures discrimination of degraded or distorted speech, discrimination in the presence of a competing message in the opposite ear, the ability to fuse incomplete or partial messages delivered to each ear into a meaningful message, and the capacity to localize sound in space when acoustic stimuli are delivered simultaneously to both ears. This testing should be done on certain patients, such as children with a reading or other learning problem and older people who seem to hear but do not comprehend.

In children with hearing loss, additional testing should include an ophthalmologic examination because many genetic causes of deafness also cause ocular abnormalities. Children with unexplained hearing loss should also have an ECG to look for long QT syndrome and possibly also genetic testing.

Amplification of sound with a hearing aid helps many people. Although hearing aids do not restore hearing to normal, they can significantly improve communication. Advances in amplification circuits provide a more natural, tonal quality to amplified sound and offer features of "smart," responsive amplification that takes into account the listening environment (eg, in noise-challenging and multi-talker environments). Physicians should encourage hearing aid use and help patients overcome a sense of social stigma that continues to obstruct use of these devices, perhaps by making the analogy that a hearing aid is to hearing as eye glasses are to seeing. Other factors that limit more widespread hearing aid use include cost and comfort issues.

All hearing aids have a microphone, amplifier, speaker, earpiece, and volume control, although they differ in the location of these components. An audiologist should be involved in selection and fitting of a hearing aid.

The best models are adjusted to a person’s particular pattern of hearing loss. For example, people with mainly high-frequency hearing loss might not benefit from simple amplification, which merely makes the garbled speech they hear sound louder; they usually need a hearing aid that selectively amplifies the high frequencies. Some hearing aids contain vents in the ear mold, which facilitate the passage of high-frequency sound waves. Some use digital sound processing with multiple frequency channels so that amplification more precisely matches hearing loss as measured on the audiogram.

Telephone use can be difficult for people with hearing aids. Typical hearing aids cause squealing when the ear is placed next to the phone handle. Some hearing aids have a phone coil with a switch that turns the microphone off and links the phone coil electromagnetically to the speaker magnet in the phone.

For moderate to severe hearing loss, a postauricular (ear-level) aid, which fits behind the pinna and is coupled to the ear mold with flexible tubing, is appropriate. An in-the-ear aid is contained entirely within the ear mold and fits less conspicuously into the concha and ear canal; it is appropriate for mild to moderate hearing loss. Some people with mild hearing loss limited to high frequencies are most comfortably fitted with postauricular aids and completely open ear canals. Canal aids are contained entirely within the ear canal and are cosmetically acceptable to many people who would otherwise refuse to use a hearing aid, but they are difficult for some people (especially older people) to manipulate.

The CROS aid (contralateral routing of signals) is occasionally used for severe unilateral hearing loss; a hearing-aid microphone is placed in the nonfunctioning ear, and sound is routed to the functioning ear through a wire or radio transmitter. This device enables the wearer to hear sounds from the nonfunctioning side, allowing for some limited capacity to localize sound. If the better ear also has some hearing loss, the sound from both sides can be amplified with the binaural CROS (BiCROS) aid.

The body aid type is appropriate for profound hearing loss. It is worn in a shirt pocket or a body harness and connected by a wire to the earpiece (the receiver), which is coupled to the ear canal by a plastic insert (ear mold).

A bone conduction aid may be used when an ear mold or tube cannot be used, as in atresia of the ear canal or persistent otorrhea. An oscillator is held against the head, usually over the mastoid, with a spring band, and sound is conducted through the skull to the cochlea. Bone conduction hearing aids require more power, introduce more distortion, and are less comfortable to wear than air conduction hearing aids. Some bone conduction aids (bone-anchored hearing aids or BAHAs) are surgically implanted in the mastoid process, avoiding the discomfort and prominence of the spring band.

Patients with advanced levels of hearing loss, including those with some residual (natural) hearing but who even with a hearing aid cannot understand more than half of the words contained in connected speech, may benefit from a cochlear implant. The surgery may result in loss of some residual hearing. However, even if residual hearing is lost, cochlear implants can greatly improve hearing, even in people who are profoundly deaf.

Cochlear Implant

Image

JACOPIN/BSIP/SCIENCE PHOTO LIBRARY

This device provides electrical signals directly into the auditory nerve via multiple electrodes implanted in the cochlea. An external microphone and processor convert sound waves to electrical impulses, which are transmitted through the skin electromagnetically from an external induction coil to an internal coil implanted in the skull above and behind the ear. The internal coil connects to electrodes inserted in the scala tympani.

Cochlear implants help with speech-reading by providing information about the intonation of words and the rhythm of speech. Many if not most adults with cochlear implants can discriminate words without visual clues, allowing them to talk on the telephone. Cochlear implants enable deaf people to hear and distinguish environmental sounds and warning signals. They also help deaf people modulate their voice and make their speech more intelligible.

Outcomes with cochlear implants vary, depending on a number of factors, including the

• Length of time between onset of hearing loss and placement of the implant (shorter duration leads to better outcomes)

• Cause of the underlying hearing loss

• Position of the implant within the cochlea

Although cochlear implants are not an option for patients who have had both acoustic nerves destroyed (eg, by bilateral temporal bone fractures or neurofibromatosis) or are born without cochlear nerves, these patients can have some hearing restored by means of brain stem implants that have electrodes connected to sound-detecting and sound-processing devices similar to those used for cochlear implants, although usually not as good. In general, children born without cochlear nerves who receive the brain stem implant tend to have more hearing restored than patients who receive it following vestibular neuroma resection. Results of the brain stem implant range from assistance with lip-reading skills to the ability to understand language without lip-reading (termed 'open-set speech' understanding).

Alerting systems that use light let people know when the doorbell is ringing, a smoke detector is sounding, or a baby is crying. Special sound systems transmitting infrared or FM radio signals help people hear in theaters, churches, or other places where competing noise exists. Many television programs carry closed captioning. Telephone communication devices are also available.

Lip-reading or speech-reading is particularly important for people who can hear but have trouble discriminating sounds. Most people get useful speech information from lip-reading even without formal training. Even people with normal hearing can better understand speech in a noisy place if they can see the speaker. To use this information the listener must be able to see the speaker’s mouth. Health care personnel should be sensitive to this issue and always position themselves appropriately when speaking to the hearing-impaired. Observing the position of a speaker’s lips allows recognition of the consonant being spoken, thereby improving speech comprehension in patients with high-frequency hearing loss. Lip-reading may be learned in aural rehabilitation sessions in which a group of age-matched peers meets regularly for instruction and supervised practice in optimizing communication.

People can gain control over their listening environment by modifying or avoiding difficult situations. For example, people can visit a restaurant during off-peak hours, when it is quieter. They can ask for a booth, which blocks out some extraneous sounds. In direct conversations, people may ask the speaker to face them. At the beginning of a telephone conversation, they can identify themselves as being hearing-impaired. At a conference, the speaker can be asked to use an assistive listening system, which makes use of either inductive loop, infrared, or FM technology that sends sound through the microphone to a patient’s hearing aid.

People with profound hearing loss often communicate by using sign language. American Sign Language (ASL) is the most common version in the US. Other forms of linguistic communication that utilize visual inputs include Signed English, Signing Exact English, and Cued Speech. Around the world, it has been estimated that there are over 300 unique sign languages, with different countries, cultures, and villages having their own unique form of sign language.

Presbycusis is sensorineural hearing loss that probably results from a combination of age-related deterioration and cell death in various components of the hearing system and the effects of chronic noise exposure.

Hearing loss usually affects the highest frequencies (18 to 20 kHz) early on and gradually affects the lower frequencies; it usually becomes clinically significant when it affects the critical 2- to 4-kHz range at about age 55 to 65 (sometimes sooner). The loss of high-frequency hearing significantly affects speech comprehension. Although the loudness of speech seems normal, certain consonant sounds (eg, C, D, K, P, S, T) become harder to hear. Consonant sounds are the most important sounds for speech recognition. For example, when “shoe,” “blue,” “true,” “too,” or “new” is spoken, many people with presbycusis can hear the “oo” sound, but most have difficulty recognizing which word has been spoken because they cannot distinguish the consonants. This inability to distinguish consonants causes affected people to often think the speaker is mumbling. A speaker attempting to speak louder usually accentuates vowel sounds (which are low frequency), doing little to improve speech recognition. Speech comprehension is particularly difficult when background noise is present.

A screening tool is often helpful for older people because many do not complain of hearing loss. One tool is the Hearing Handicap Inventory for the Elderly–Screening Version, which asks

• Does a hearing problem cause you to feel embarrassed when you meet people?

• Does a hearing problem cause you to feel frustrated when talking to a family member?

• Do you have difficulty hearing when someone whispers?

• Do you feel handicapped by a hearing problem?

• Does a hearing problem cause you difficulty when visiting friends, relatives, or neighbors?

• Does a hearing problem cause you to attend religious services less often than you would like?

• Does a hearing problem cause you to have arguments with family members?

• Does a hearing problem cause you difficulty when listening to the television or radio?

• Do you feel that any difficulty with your hearing hampers your personal or social life?

• Does a hearing problem cause you difficulty when in a restaurant with relatives or friends?

Scoring is “no” = 0 points, “sometimes” = 2 points, and “yes” = 4 points. Scores > 10 suggest significant hearing impairment and necessitate follow-up.