Children diagnosed for Usher Syndrome who were born hard of hearing or whose hearing deteriorated during childhood or at adult age, have a (progressive) perceptive loss of hearing.

  • Perceptive deafness literally means: observational deafness, also called sensorineural deafness. The sound perception in the cochlea does not function (properly).
  • Perceptive loss of hearing in both ears is called binaural perceptive loss of hearing.

The perceptive loss is characterised by more loss at the high frequencies. Usually, low tones can usually be heard reasonably well. Distortion of sound occurs. Because of this, spoken words are heard but not always correctly understood. Perceptive loss often goes together with recruitment. Here soft sounds are hardly perceived and at the same time the person is over-sensitive for loud noises. People with this type of loss of hearing cannot properly distinguish sounds and may have problems localising and recognising sounds. People with perceptive loss of hearing have difficulty with following a conversation in a room with a lot of ambient noise.

These people have worn hearing aids in both ears as from a young age and language and speech have usually developed successfully. Sometimes some extra support is needed from a speech therapist. Dutch sign language can help young children to better express themselves, but most learn how to make use of lip reading.

Many Ushers who are hard of hearing have high speech scores in the speech audiometry tests. These often are higher than expected in view of the loss of hearing. The effort and concentration required for this are often not measured.


Another additional complaint that may occur in case of fast increasing loss of hearing or very serious loss of hearing is tinnitus (ringing ears). This is often caused by the (fast) deterioration of hearing.

The hyperacusis or tinnitus can disturb the understanding of speech, especially in situations with ambient noise. Then understanding speech requires extra effort. This effort may lead to fatigue and stress complaints. Not being able to properly understand people may result in misunderstandings and mutual irritations. Eventually, the consequence of this may be that the deaf person starts to avoid contacts and this may cause depression.

Additional information about hyperacusis and tinnitus is to be found on the websites, en


In the sound clips given below you can hear a conversation. This gives some impression of how people with perceptive loss of hearing hear.

In the link below the poor understanding of speech has been visualised.

More sound clips can be heard on the website of The type of loss of hearing in case of damage caused by noise can be compared with perceptive loss of hearing with people suffering from Usher Syndrome. Usually people with Usher Syndrome lose their hearing in the high tones of 60 dB or higher.


With people suffering from Usher Syndrome who are born hard of hearing the loss of hearing is quite stable.
Some people have progressive loss of hearing and their hearing deteriorates every 5 to 10 years by 5-10 decibels. Eventually, these people will have lost so much of their hearing, that hearing aids will no longer be of any help. Since the year 2016, deafblind adults may be eligible for two cochlear implants, which makes it possible for them to understand speech and to localise sound again.