Data from 24 previously published ADPEAF families with mutations in the leucine-rich, glioma inactivated 1 gene (LGI1) were analyzed, in a study at Columbia University, New York. Penetrance is 67%, it tends to be greater in families with more affected individuals, does not differ with gender, and may increase with advancing generation. [1]

COMMENT. Autosomal dominant (AD) partial epilepsy with auditory features, also known as AD lateral temporal epilepsy, is an idiopathic focal epilepsy syndrome manifested by auditory symptoms (humming, buzzing, or ringing, volume changes, specific songs or voices) or receptive aphasia. Ictal receptive aphasia is a sudden inability to understand language without general confusion. Mutations in the LGI1 gene located on chromosome 10 occur in approximately 50% of families with ADPEAF, but not in families with other familial temporal lobe epilepsies.

Symptomatic temporal lobe auditory seizures. Many examples of seizures secondary to temporal lobe tumor and manifested by auditory illusions and hallucinations are included in the classic book by Penfield W, and Jasper H [2]. One patient, a housewife of 43 years, complained of ringing in the ears and of hearing voices or music, a song she had heard previously, an hallucination sometimes she described as a dream. Another, a girl aged 16 years, heard a lullaby that her mother used to sing to her as an infant. In some attacks she complained of a change in volume or fading away of sounds, considered an illusional seizure. Stimulation of the cortex in the superior gyrus of the left temporal lobe illicited hallucinations of voices, saying words, repeated music (accompanied by humming), and “There was singing” response. Penfield referred to these as “psychical seizures.” In children, temporal lobe seizures often begin with a sense and look of fear, the child clinging to mother, although unable to describe a possible associated hallucination.