LRTOMT (leucine rich transmembrane and O-methyltransferase domain containing) is a dual-function protein essential for auditory function. The gene encodes two protein products through alternative reading frames: LRTOMT1 and LRTOMT2, a putative O-methyltransferase 1. While LRTOMT catalyzes O-methylation of catecholamine neurotransmitters, its critical role in hearing is independent of enzymatic activity. LRTOMT functions as a component of cochlear hair cell mechanotransduction machinery, specifically involved in assembling the asymmetric tip-link complex and transporting TMC1 and TMC2 proteins into mechanically sensitive stereocilia 2. Biallelic mutations in LRTOMT cause autosomal recessive nonsyndromic hearing loss (deafness DFNB63), with clinical presentation ranging from moderate progressive to profound sensorineural hearing loss 1, 2. The c.242G>A mutation shows particularly high prevalence in Moroccan populations, making LRTOMT the second most common genetic cause of congenital deafness after GJB2 in affected regions 3. Compound heterozygous mutations have been documented in nonconsanguineous families, and novel variants continue to be identified across diverse populations 2, 4, 5.